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Comprehensive
Textbook of Echocardiography
Vol.
Comprehensive
Textbook of Echocardiography
Editor
Navin C Nanda MD
Jaypee-Highlights.
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2014, Jaypee Brothers Medical Publishers
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Comprehensive Textbook of Echocardiography (Vol. 2)
First Edition: 2014
ISBN978-93-5090-634-7
Printed at:
Dedicated to
My late parents
Balwant Rai Nanda MD and Mrs Maya Vati Nanda
My wife
Kanta Nanda MD
Our children
Nitin Nanda, Anita Nanda Wasan MD and Anil Nanda MD
Their spouses Sanjeev Wasan MD and Seema Tailor Nanda, and
our grandchildren Vinay and Rajesh Wasan, and Nayna and Ria Nanda
Contributors
Masood Ahmad M
D FRCP (C) FACP FACC
FAHA FASE
Division of Cardiology
Department of Internal Medicine
University of Texas Medical Branch
Galveston Texas, USA
Mohammad Al-Admawi MD
King Faisal Specialist Hospital and
Research Center
Heart Center
Riyadh, Saudi Arabia
Bader Almahdi MD
Professor of Medicine
University at Buffalo School of Medicine
and Biological Sciences
New York, USA
Division of Cardiology
Geisinger-Community Medical
Center, and The Wright Center for
Graduate Medical Education
Scranton, Pennsylvania, USA
Charles E Beale MD
Department of Medicine
Division of Cardiovascular Diseases
Stony Brook University Medical Center
Stony Brook, New York, USA
Roy Beigel MD
The Heart Institute, Cedars Sinai Medical
Center, Los Angeles, California, USA
The Leviev Heart Center Sheba Medical
Center, Affiliated to the Sackler
School of Medicine
Tel Aviv University, Tel Aviv, Israel
Steven Bleich MD
Department of Medicine
Division of Internal Medicine
University of Alabama at Birmingham
Birmingham, Alabama, USA
O Julian Booker MD
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama, USA
Division of Cardiology
Department of Medicine
University of Texas Medical Branch
Galveston, Texas, USA
Luis Bowen MD
Department of Medicine
University of Alabama at Birmingham
Birmingham, Alabama, USA
Aditya Bharadwaj MD
Gerald Buckberg MD
Department of Cardiology
Loma Linda University and
VA Medical Centers
Loma Linda, California, USA
Michael J Campbell MD
Assistant Professor
Division of Pediatric Cardiology
Seattle Childrens Hospital and
University of Washington
Seattle, Washington, USA
Department of Pediatrics
Division of Pediatric Cardiology
Duke University Medical Center
Durham, North Carolina, USA
Piers Barker MD
Nicole Bhave MD
FRCP FACC
Department of Pediatrics
Division of Pediatric Cardiology
Duke University Medical Center
Durham, North Carolina, USA
Professor Emeritus
Division of Cardiology
UC-Irvine School of Medicine
Irvine, California, USA
Ricardo Benenstein MD
viii
Leon H Charney
Daniel Forsha MD
Division of Cardiology
New York University Medical Center
New York, New York, USA
Department of Cardiology
Second University of Naples:
Monaldi Hospital, Naples, Italy
Department of Pediatrics
Division of Pediatric Cardiology
Duke University Medical Center
Durham, North Carolina, USA
Farooq A Chaudhry M
D FACP FACC
David Daly MD
FASE FAHA
Professor of Medicine
Director, Echocardiography Laboratories
Associate Director, Mount Sinai Heart
Network, Icahn School of Medicine at
Mount Sinai, Zena and Michael A Wiener
Cardiovascular Institute and
Marie-Jose and Henry R Kravis Center
for Cardiovascular Health
New York, New York, USA
Preeti Chaurasia MD
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama
Krishnaswamy Chandrasekaran MD
Mayo Clinic, Scottsdale, Arizona, USA
Rochester, Minnesota, USA
Michael Chen MD
University of Washington
Seattle, Washington DC, USA
HK Chopra MD
Moolchand City Hospital
New Delhi, India
Department of Medicine
University of Alabama at Birmingham
Birmingham, Alabama, USA
Hisham Dokainish M
D FRCPC
Associate Professor of Medicine
McMaster University
Director of Echocardiography and
Medical Diagnostic Units
Hamilton Health Sciences
Hamilton, Ontario, Canada
Maximiliano German
Amado Escauela MD
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama, USA
Bahaa M Fadel MD
King Faisal Specialist Hospital and
Research Center
Heart Center
Riyadh, Saudi Arabia
Luna Gargani MD
Institute of Clinical Physiology
National Research Council
Pisa, Italy
Division of Cardiology
Fondazione Cardiocentro Ticino
Lugano, Switzerland
Honorary Consultant
Imperial and King's Colleges, London, UK
Francesco Faletra MD
Francesco Ferrara MD
David Cosgrove MD
Leon J Frazin MD
Department of Cardiology
Loma Linda University and
VA Medical Centers, Loma Linda
California, USA
Cecil Coghlan MD
Gary P Foster MD
Contributors
ix
Donald Hagler MD
Mayo Clinic
Rochester, Minnesota, USA
Stephanie El-Hajj MD
Arzu Ilercil MD
Nina Ghosh MD
Kamran Haleem MD
Trevor Jenkins MD
Edward Gill MD
Professor of Medicine and
Cardiology, University of Washington
Seattle, Washington DC, USA
Willem Gorissen
Clinical Market Manager
Toshiba Medical Systems Europe
Zoetermeer, The Netherlands
Rakesh Gupta MD
JROP Healthcare
New Delhi, India
Fadi G Hage MD
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama, USA
Section of Cardiology, Birmingham
Veterans, Administration Medical Center
Birmingham, Alabama, USA
Yale University
New Haven, Connecticut, USA
Dan G Halpern MD
St Lukes-Roosevelt Hospital Center
Columbia University, College of
Physicians and Surgeons
New York, New York, USA
Christine Henri MD
Department of Cardiology
Heart Valve Disease Clinic
CHU Sart Tilman, University of
Lige, Belgium
Julien IE Hoffman MD
Department of Pediatrics
University of California
San Francisco, California, USA
Brian D Hoit MD
Director of Echocardiography
Harrington Heart & Vascular Center
University Hospitals of Cleveland
Texas, USA
Madhavi Kadiyala MD
Saint Francis Hospital, Roslyn
New York, USA
Arshad Kamel MD
Department of Medicine
University of Alabama at Huntsville
Huntsville, Alabama, USA
Abdallah Kamouh MD
University of Buffalo
Buffalo, New York, USA
Kanwal K Kapur MD
DM Cardiology, Sr Consultant and Chief
Noninvasive Cardiology
Indraprastha Apollo Hospitals
New Delhi, India
Department of Noninvasive Cardiology
Indraprastha Apollo Hospitals
New Delhi, India
Jarosaw D Kasprzak MD
Chair and Department of Cardiology
Biegaski Hospital
Medical University of Lodz
Lodz, Poland
Arthur J Labovitz MD
Gerald R Marx MD
Professor of Medicine
Cardiology Section
Director of Echocardiography
Temple University School of Medicine
Parkinson Pavilion, Suite
North Broad Street, Philadelphia
Pennsylvania, USA
Professor of Medicine
Chair, Department of
Cardiovascular Sciences
University of South Florida
Tampa, Florida, USA
Associate Professor
Harvard School of Medicine
Senior Associate Cardiology
Boston Childrens Hospital
Boston, Massachusetts, USA
Jennifer K Lang MD
Wilson Mathias Jr MD
University at Buffalo
Buffalo, New York, USA
Tuba Kemalolu z MD
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama, USA
Anant Kharod MD
Department of Medicine
University of Alabama at Birmingham
Birmingham, Alabama, USA
Jennifer Kiessling MD
Division of Cardiovascular
Diseases, University of
Alabama at Birmingham
Birmingham, Alabama, USA
Allan L Klein M
D FRCP(C) FACC
Roberto M Lang MD
University of Chicago
Medical Center
Chicago, Illinois, USA
Steve W Leung MD
Assistant Professor of Medicine
Division of Cardiovascular Medicine
University of Kentucky
Lexington, Kentucky, USA
FAHA FASE
Angele A A Mattoso MD
Heart Institute (InCor)
The University of So Paulo School of
Medicine, So Paulo, Brazil and
Santa Izabel Hospital, Salvador, Bahia
Anjlee M Mehta MD
Fellow, Division of Cardiology
Dartmouth-Hitchcock Heart and
Vascular Center
Lebanon, New Hampshire, USA
Sachin Logani MD
Professor of Medicine
State University of New York
Stony Brook Director
Non Inavasive Cardiac Imaging Director
Echocardiography Diretor
Valve Center, Stony Brook Medicine
Stony Brook, New York, USA
Itzhak Kronzon M
D FASE FACC FACP
Department of Medicine
Division of Cardiovascular Diseases
Stony Brook University
Medical Center, Stony Brook
New York, USA
Judy R Mangion MD
Division of Cardiovascular Medicine
Brigham and Womens Hospital
Harvard Medical School
Francis Street, Boston
Massachusetts, USA
FESC FAHA
Professor of Cardiology
Hofstra University
North Shore LIJ, School of Medicine
Chief of Noninvasive Cardiac Imaging
Lenox Hill Hospital
Noninvasive Cardiology
New York, New York, USA
CN Manjunath MD DM
Director, Professor and Head
Department of Cardiology
Sri Jayadeva Institute of Cardiovascular
Sciences and Research
Bannergutta Road
Bengaluru, Karnataka, India
Institute of Critical
Care and Anesthesia
Medanta The Medicity
Gurgaon, Haryana, India
Andrew P Miller MD
Cardiovascular Associates
Birmingham, Alabama, USA
Contributors
Dilbahar S Mohar MD
Ryozo Omoto MD
Division of Cardiology
UC-Irvine School of Medicine
Irvine, California, USA
Caroline Morbach MD
Yale University
New Haven, Connecticut, USA
Consultant Cardiologist
Head, Non-Invasive Cardiology Lab
King Abdulaziz Cardiac CenterRiyadh
Health AffairsMinistry of National Guard
Kingdom of Saudi Arabia
Nagaraja Moorthy MD DM
Hoda Mojazi-Amiri MD
Assistant Professor
Department of Cardiology
Sri Jayadeva Institute of Cardiovascular
Sciences and Research
Bengaluru, Karnataka, India
Hirohiko Motoki MD
Cardiovascular Research
Imaging Fellow, Cleveland Clinic
Foundation, Cleveland, Ohio, USA
Bernhard Mumm
President and COO
TomTec Imaging Systems
GmbH, Edisonstr
Unterschleissheim, Germany
Navin C Nanda MD
Distinguished Professor of Medicine and
Cardiovascular Disease and
Director, Heart Station/Echocardiography
Laboratories, University of Alabama at
Birmingham and the University of
Alabama Health Services Foundation
The Kirklin Clinic, Birmingham
Alabama, USA, President, International
Society of Cardiovascular Ultrasound
xi
MRCP
Shyam Padmanabhan MD
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama, USA
Ramdas G Pai MD
Professor of Medicine
Loma Linda University
Medical Center
Loma Linda, California, USA
Natesa G Pandian MD
Professor, Tufts University School of
Medicine, Director, Heart Valve Center
Co-Director, Cardiovascular
Imaging Center
Director, Cardiovascular Ultrasound
Research, Tufts Medical Center
Boston, Massachusetts, USA
Satish K Parashar MD
Metro Heart Institute
New Delhi, India
Anita Radhakrishnan MD
Fellow, Division of Cardiology
Allegheny General Hospital
Pittsburgh, Pennsylvania, USA
Peter S Rahko MD
Professor of Medicine
Division of Cardiovascular Medicine
Department of Medicine, University of
Wisconsin School of Medicine and Public
Health, Madison, Wisconsin, USA
Rajesh Ramineni MD
University of Texas Medical Branch
Galveston, Texas, USA
JRTC Roelandt MD
Professor of Cardiology
Honorary Chairman, Thoraxcentre
Erasmus University Medical
Centre, Rotterdam
The Netherlands
Lindsay Rogers MD
Department of Pediatrics
Division of Pediatric Cardiology
Vanderbilt University Medical Center
Nashville, Tennessee, USA
Ashvin K Patel MD
David A Parra MD
xii
Teresa Sevilla MD
Robert J Siegel MD
Emanuele Romeo MD
James Seward MD
Department of Cardiology
Second University of Naples
Monaldi Hospital, Naples, Italy
Mayo Clinic
Rochester, Minnesota, USA
Utpal N Sagar MD
Advanced Cardiovascular Imaging Fellow
Heart and Vascular Institute
Department of Cardiovascular Medicine
Cleveland, Ohio, USA
Giovanni Di Salvo MD
King Faisal Specialist
Hospital and Research Center
Heart Center
Riyadh, Saudi Arabia
Benoy Nalin Shah BSc
(Hons) MBBS MRCP
Mark V Sherrid MD
Nelson B Schiller MD
Professor of Medicine
University of California
San Francisco
UCSF Division of Cardiology
Parnassus Avenue
San Francisco, California, USA
Siddharth Singh MD MS
Division of Cardiovascular Disease
University of Alabama at Birmingham
Birmingham, Alabama, USA
Chittur A Sivaram MD
David Ross Boyd Professor
Vice Chief of Cardiovascular Section
Associate Dean for Continuing
Professional Development
University of Oklahoma
Health Sciences Center
Oklahoma City, Oklahama, USA
Sushilkumar K Sonavane MD
Assistant Professor
Cardiopulmonary Radiology
University of Alabama at Birmingham
Department Radiology
Birmingham, Alabama, USA
Vincent L Sorrell MD
Peter Sidarous MD
Jonathan H Soslow MD
Research Associate
UC-Irvine School of Medicine
Irvine, California, USA
Department of Pediatrics
Division of Pediatric Cardiology
Vanderbilt University Medical Center
Nashville, Tennessee, USA
Khadija Siddiqui DO
Division of Cardiology
Department of Medicine
University of Texas Medical Branch
Galveston, Texas, USA
Contributors
Sharath Subramanian MD
George Thomas MD
Department of Cardiology
Saraf Hospital, Kochi, Kerala, India
Lissa Sugeng MD
Wendy Tsang MD
Hospital Clnico
San Carlos
Madrid, Spain
Associate Professor
Director of Yale Echo Lab and
YRCG Echo Corelab
Section of Cardiovascular Medicine
Division of Medicine
Yale University School of Medicine
New Haven, Connecticut, USA
Aylin Sungur MD
Jeane M Tsutsui MD
Leon Varjabedian MD
Karina Wierzbowska-Drabik MD
Padmini Varadarajan MD
Department of Cardiology
Loma Linda University and VA Medical
Centers, Loma Linda, California, USA
Azhar Supariwala MD
Mahdi Veillet-Chowdhury MD
Division of Cardiology
St Lukes-Roosevelt Hospital Center
New York, New York, USA
Department of Cardiology
Loma Linda University and VA
Medical Centers
Loma Linda, California, USA
University of Buffalo
Buffalo, New York, USA
Pooja Swamy MD
Victor Vacanti MD
Colette Veyrat MD
Centre National de la Recherche
Scientifique Honorary Researcher
Department of Cardiovascular Medicine
LInstitut Mutualiste de Montsouris
Boulevard Jourdan, Paris Cedex, France
University of Buffalo
Buffalo, New York, USA
Timothy D Woods MD
Associate Professor of
Medicine and Radiology
Medical College of Wisconsin
Cardiology Division
Milwaukee, Wisconsin, USA
xiii
Preface
Monumental strides have occurred in the evolution of echocardiography since its first introduction in the 1950s.
It began with A-mode and M-mode echocardiography which progressed to real time two-dimensional echocardiography
in the 1970s after a hiatus of several years. This development completely revolutionized the field of noninvasive cardiac
imaging; and within a few years of its introduction, there were hardly any cardiology divisions in any hospital anywhere in
the world which did not own an ultrasound machine. The next few years saw the development of continuous and pulsed
wave Doppler and color Doppler flow imaging which provided assessment of cardiac hemodynamics to supplement
the structural information obtained using two-dimensional echocardiography. Other advances rapidly followed
or occurred concurrently. These included stress echocardiography, transesophageal echocardiography, contrast
echocardiography and tissue Doppler and velocity vector imaging. More recently, further innovations were introduced
such as live/real time three-dimensional echocardiography and both two-and three-dimensional speckle tracking
echocardiography which have obviated some of the limitations of the previous techniques and have further enhanced
the clinical usefulness of echocardiography. To this day, echocardiography represents the most useful and most costeffective noninvasive modality available for the assessment of various cardiac disease entities. The development of
allied noninvasive technologies like magnetic resonance imaging and computed tomography has further added to
the information provided by echocardiography and are useful and important additions to the armamentarium of the
cardiologists and other patient care providers in the comprehensive assessment and management of cardiac disease.
The aim of the current book is to provide an overview of the subject of clinical echocardiography as it is practiced
to-day. Given the many advances that have not only been recently introduced but are also ongoing in this field it would
be very difficult for anyone to realistically come up with a comprehensive book on echocardiography but an attempt has
been made to cover as many topics as possible in this book. In addition, the supplementary information provided by
magnetic resonance imaging and computed tomography is also included in this book.
The book consists of a total of 85 chapters organized into seven sections. The first section deals with the basics of
ultrasound, Doppler, speckle tracking, three-dimensional echocardiography and instrumentation. A short history
of echocardiography and Doppler are also included in this section. The second section consists of various aspects
of echocardiography and ultrasound examination. M-mode and two- and three-dimensional transthoracic and
transesophageal examination, nonstandard planes, various aspects of Doppler assessment including tissue Doppler,
velocity vector and speckle tracking imaging, assessment of endothelial function, contrast echocardiography for
evaluation of left ventricular endocardial border opacification and myocardial perfusion, transpharyngeal echo,
epiaortic echocardiography and both intracardiac and intravascular ultrasound are dealt with in this section. In
addition, examination with a small hand-held ultrasound system, peripheral ultrasound, echocardiographic artifacts,
quantification techniques in echocardiography and echocardiography training form a part of this section. Valvular heart
disease is covered in the next section. It deals with evaluation of mitral valve disease, mitral regurgitation, aortic stenosis
including assessment of low gradient stenosis with preserved left ventricular function, aortic regurgitation, aortic disease,
tricuspid and pulmonary valves, pulmonary hypertension, infective endocarditis and prosthetic valves. Rheumatic heart
disease is also included in this section. Section 4 deals with two- and three-dimensional echocardiographic assessment
of systolic and diastolic function of both left and right ventricles. Newer aspects of structure and function to assess cardiac
motion, evaluation of left atrial function, ventricular assist devices, pacemakers and intracardiac defibrillators and use
of echocardiography for the assessment of cardiac hemodynamics and guidance of therapy are also included in this
section. The next section contains chapters covering ischemic heart disease, coronary arteries and coronary flow reserve,
xvi
different aspects of stress echocardiography including three-dimensional stress echocardiography, obstructive and
non-obstructive cardiomyopathies, differentiation of ischemic and nonischemic cardiomyopathy, pericardial disorders
and tumors and masses. Section 6 deals with congenital heart disease and consists of chapters on fetal cardiac imaging,
M-mode and two- and three-dimensional assessment of pediatric congenital heart disease, ventricular function, adult
congenital heart disease and acquired heart diseases in childhood. The final section in the book, Section 7, covers
systemic diseases, life-threatening conditions, echocardiography in women and the elderly, echocardiography for the
electrophysiologist and lung ultrasound. A separate chapter assesses the future of echocardiography and ultrasound.
Lastly, two chapters cover the allied techniques of magnetic resonance imaging and cardiac computed tomographic
imaging. A very large number of echocardiographic images and other figures illustrate most of the chapters of the book
and six DVDs contain numerous movie clips to supplement the images. These represent a major highlight of the book.
All chapters in this book are written by well-known experts in the field of echocardiography and ultrasound. Because
of the large number of contributors, some overlap of content and chapters do exist in the book. This has been deliberately
not excluded because it provides a different perspective to the reader and also serves to reinforce important concepts
and echocardiographic findings.
Navin C Nanda MD
Acknowledgments
I am most grateful to all the contributors from different countries of the world who have taken valuable time off from
their busy schedule to prepare chapters for this book. I am also grateful to the faculty, clinical and research fellows,
medical residents, and observers, both past and present, from our institution who have directly or indirectly helped
in the preparation of this book. Special mention needs to be made of Kunal Bhagatwala, Nidhi M Karia, Steven Bleich,
Aylin Sungur, Tuba Kemalolu z, Kruti Jayesh Mehta, Maximiliano German Amado Escauela and Ming Hsuing
for their invaluable help. I wish to express my thanks to the International Society of Cardiovascular Ultrasound and
the Indian Academy of Echocardiography for agreeing to have the book under their aegis. Special thanks to all the
members of the Indian Academy of Echocardiography including the current President Dr ST Yavagal as well as Drs
Satish Parashar, HK Chopra and Rakesh Gupta for their unstinting support of this project. I especially appreciate the
constant support and encouragement of Shri Jitendar P Vij (Group Chairman) and Mr Ankit Vij (Managing Director) of
M/s Jaypee Brothers Medical Publishers (P) Ltd, New Delhi, India, in helping publish this book and also all their
associates particularly Ms Chetna Malhotra Vohra (Senior ManagerBusiness Development) and Ms Saima Rashid
(Development Editor) who have been prompt, efficient and most helpful. I also deeply appreciate the help of Lindy
Chapman, Administrative Associate at the University of Alabama at Birmingham, who provided excellent editorial and
secretarial assistance, and Diane Blizzard, Office Associate, for her help. Last but not least, I appreciate the patience,
understanding and support of my wife, Kanta Nanda.
Contents
xix
Contents
Volume 1
History of Ultrasound 4
The Development of Clinical Cardiac Ultrasound: A-Mode and M-Mode Echocardiography 4
Two-Dimensional Echocardiography 8
Conventional Doppler Ultrasound 9
Color Doppler Ultrasound 11
Contrast Echocardiography 11
Transesophageal Echocardiography 13
Tissue Doppler and Speckle Tracking Imaging 14
Three-Dimensional Echocardiography 14
Perspective 19
24
Colette Veyrat
The Preflow Doppler Era: Paucity of Existing Noninvasive Tools 25
Explosive Emergence of the Flow Concept, an Indispensable Mutation from
Pressure Measurements, which Prepared the Doppler Flow Era 27
Return to the Doppler Technique in Search of a Noninvasive Tool
Documenting the Flow Concept 28
3. Basics of Ultrasound
55
General Physics 55
Imaging by Ultrasound 57
Image Optimization and Equipment 60
Artifacts 61
Doppler Ultrasound 63
Doppler in Cardiology 65
Doppler Instrumentation 66
Continuous Wave Doppler 68
Pulsed Wave Doppler 69
Color Doppler 71
65
xx
Power Doppler 71
Tissue Doppler 72
The Doppler Methodology 72
Information Derived from Doppler 73
5. Basics of 3D Ultrasound
74
Evolution of 3D Echocardiography 74
Transducer Technology 76
Beam Forming 77
Rendering 78
Limitations in 3D Image Quality 80
3D Echocardiography Quantification 81
87
99
119
Contents
xxi
132
164
188
Navin C Nanda, Aylin Sungur, Kunal Bhagatwala, Nidhi M Karia, Tuba Kemalolu z
224
3D Technology 240
3D Examination Protocol 241
Left Parasternal Approach 244
Apical Approach 244
Subcostal Approach 244
Suprasternal Approach 244
Supraclavicular Approach 244
Right Parasternal Approach 246
Color Doppler Imaging 248
Advantages/Disadvantages of 3D Echocardiography 262
240
xxii
268
History 268
Methods for Data Acquisition 268
Left Ventricular Assessment 270
Reproducibility 272
Regional LV Function 276
Aortic Regurgitation 280
Aortic Annulus 280
Mitral Stenosis 280
Mitral Regurgitation 282
Tricuspid Valve Disease 283
Pulmonic Valve Disease 284
Advances in Pediatric and Fetal Cardiac Pathologies 285
291
JRTC Roelandt
299
325
Contents
xxiii
349
Hisham Dokainish
Technical Considerations 349
Development of Tissue Doppler Imaging 350
Current Clinical Uses of TD Imaging 350
360
380
Michael J Campbell, David A Parra, Daniel Forsha, Piers Barker, Jonathan H Soslow
416
441
449
xxiv
480
487
Stephanie El-Hajj, Navin C Nanda, Kunal Bhagatwala, Nidhi M Karia, Fadi G Hage
Technique and Recognition of Vessels 487
Application 495
507
515
Contents
xxv
577
Ahmad S Omran
638
643
655
xxvi
663
705
732
750
765
Contents
xxvii
826
Overview 826
Echocardiographic Assessment of Mitral Stenosis 826
Echocardiographic Assessment of Mitral Regurgitation 847
Assessment of Severity of Mitral Regurgitation 863
880
Etiology 880
Mechanisms 884
Severity of Mitral Regurgitation 885
Mitral Regurgitation Consequences 889
Sequential Evaluation of Chronic Asymptomatic Mitral Regurgitation 890
Feasibility of Mitral Valve Repair 892
Role of Exercise Echocardiography 892
896
919
930
xxviii
945
Martin G Keane
967
Leon J Frazin
The Anatomical Relationship of the Aorta and Esophagus 967
Imaging the Aorta with Transesophageal Echocardiography 967
984
1031
Epidemiology 1031
Pulmonary Stenosis 1032
Pulmonary Regurgitation 1036
Echocardiographic Evaluation 1037
Ross Procedure 1038
Postpulmonary Valve Surgery: Monitoring Sequelae 1039
Other Complementary Techniques for Evaluation of Pulmonary Valves 1040
1042
Javier Lpez, Teresa Sevilla, Jos Alberto San Romn, Isidre Vilacosta,
Maximiliano German Amado Escanuela, Kunal Bhagatwala, Nidhi M Karia, Navin C Nanda
1063
Michele D' Alto, Francesco Ferrara, Emanuele Romeo, Anna Agnese Stanziola, Eduardo Bossone
Conventional Echocardiography 1063
Nonconventional Echocardiography 1070
Diagnostic Algorithm in Pulmonary Hypertension 1073
Contents
xxix
1080
Aditya Bharadwaj, Pooja Swamy, Gary P Foster, Padmini Varadarajan, Ramdas G Pai
1094
Volume 2
1115
1124
Hisham Dokainish
Integrating Echocardiographic Variables for Accurate Diagnosis of Diastolic Function 1130
Novel Imaging Techniques and Future Directions 1131
1134
xxx
1149
Aasha S Gopal
3D Quantitation of the Left Ventricle 1149
3D Quantitation of the Right Ventricle 1165
1176
1210
1222
Peter S Rahko
Anatomy 1255
Physiology 1256
Functional Assessment 1257
Left Atrial Pathophysiology 1259
1255
Contents
xxxi
1264
1289
1306
1323
1328
xxxii
1337
1348
1369
Rohit Gokhale, Manreet Basra, Victor Vacanti, Steven J Horn, Aylin Sungur,
Robert P Gatewood Jr, Navin C Nanda
Cardiomyopathies 1369
Dilated Cardiomyopathy (DCM) 1370
Secondary Findings in Dilated Cardiomyopathy 1372
The Role of Echocardiography in Optimizing Heart Failure 1376
Echocardiography in Assessing Ventricular Remodeling 1379
Findings in Dilated Cardiomyopathy Based on Etiology 1379
Restrictive Cardiomyopathy 1397
Other Infiltrative Cardiomyopathies 1405
Infectious and Metabolic Cardiomyopathies 1405
Carcinoid Heart Disease 1407
1435
Contents
xxxiii
1462
Leon Varjabedian, Jennifer K Lang, Abdallah Kamouh, Steven J Horn, Tuba Kemalolu z
Aylin Sungur, Kruti Jayesh Mehta, Kunal Bhagatwala, Nidhi M Karia
Maximiliano German Amado Escauela, Robert P Gatewood Jr, Navin C Nanda
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Aarti H Bhat
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Part 2: Left-to-Right Shunts: Atrial Septal Defect, Ventricular Septal Defect, Patent Ductus Arteriosus, and
Aortopulmonary Window 1582
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Reema Chugh
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David Cosgrove
1998
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SECTION 4
Left and Right Ventricles,
Left Atrium, Hemodynamics
Chapters
Chapter 51 M-Mode and Two-Dimensional Echocardiographic
Assessment of Left Ventricular Systolic Function
Chapter 52 How to Assess Diastolic Function
Chapter 53 Evaluation of the Right Ventricle
Chapter 54 Three-Dimensional Echocardiographic
Assessment of LV and RV Function
Chapter 55 Newer Aspects of Structure/Function to Assess
Cardiac Motion
Chapter 56 Echocardiography in Assessment of Complications
Related to Permanent Pacemakers and Intracardiac
Defibrillators
CHAPTER 51
M-Mode and Two-Dimensional
Echocardiographic Assessment of Left
Ventricular Systolic Function
Anjlee M Mehta, Navin C Nanda
Snapshot
INTRODUCTION
The evaluation of left ventricular systolic function by
echocardiography has undergone many recent advance
ments. Assessment of ejection fraction as a surrogate for
left ventricular systolic function is one of the primary
clinical questions for which echocardiograms are obtained.
A review of methodologies for determining ejection
fraction and/or left ventricular function by M-mode and
two-dimensional (2D) echocardiography allows for a
better understanding of advantages, disadvantages, and
appropriate indications for echocardiographic evaluation
of left ventricle (LV) systolic function.
ArterialVentricular Coupling
1116
Fig. 51.1: Seventeen-segment model with correspondence to coronary artery distribution. (LAD: Left anterior descending artery;
LCX: left circumflex artery; RCA: Right coronary artery).
Source: Reproduced with permission from Pereztol-Valdes O, Candell-Riera J, et al. Correspondence between left ventricular
17 myocardial segments and coronary arteries. Eur Heart J. 2005(26):263743.
Chapter 51: M-Mode and Two-Dimensional Echocardiographic Assessment of Left Ventricular Systolic Function
1117
Fig. 51.2: Typical distributions of the right coronary artery (RCA), the left anterior descending (LAD), and the circumflex (CX) coronary
arteries. The arterial distribution varies between patients. Some segments have variable coronary perfusion. J Am Soc Echocardiogr.
2005;18(12):144063.
1118
Fig. 51.3: Apical four-chamber view of a normal heart at endsystole and end-diastole. In method A, left ventricular (LV) minor
axis D is measured at end-systole and end-diastole at the midventricular cavity level. The left ventricular major axis is measured
from the apex of the left ventricle to the base of the mitral valve.
In method B, measurements of the regional left ventricular minor
axes, D1, D2, and D3 are measured at three equidistance points
at the upper, middle, and lower third of the left ventricular cavity at
end-systole and end-diastole of the same cardiac cycle. The major
axis L is measured as before. Directions I, L, R, and S are inferior,
left, right, and superior, respectively. (LA: Left atrium; RA: Right
atrium; RV: Right ventricular).
Source: Reproduced with permission from Baran AO, et al.
Ejection fraction determination without planimetry by two-dimensional echocardiography: a new method. J Am Coll Cardiol.
1983;1:14718.
Chapter 51: M-Mode and Two-Dimensional Echocardiographic Assessment of Left Ventricular Systolic Function
1119
Fig. 51.6: Use of Simpsons rule. Using this rule, the volume of left
ventricle is usually calculated by approximating areas along the
apical axis by circles and employing axial integration.
Source: Reproduced with permission from Ghosh A, Nanda
NC, Maurer G. Three-dimensional reconstruction of echocardiographic images using the rotation method. Ultrasound Med Biol.
1982;8(6);65561.
DOPPLER ECHOCARDIOGRAPHIC
METHODS OF ASSESSMENT OF LEFT
VENTRICULAR FUNCTION
Fig. 51.5: Two-dimensional measurements for volume calculations using biplane method of disks (modified Simpsons rule) in
apical four-chamber (A4C) and apical two-chamber (A2C) views
at left ventricular end-diastole (LV EDD) and at left ventricular endsystole (LV ESD). Papillary muscles should be excluded from the
cavity in the tracing.
Source: Reproduced with permission from Lang RM, Bierig M,
Devereux RB, et al. Recommendations for chamber quantification: a report from the American Society of Echocardiographys
Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European
Association of Echocardiography, a branch of the European Society of Cardiology. J Am SocEchocardiogr. 2005;18(12):144063.
1120
Fig. 51.7: Teis Index. Time a is the interval between cessation and
onset of mitral inflow. It includes isovolumic contraction time (ICT),
ejection time (ET), and isovolumic relaxation time (IRT). Left ventricular ejection time b is the duration of the left ventricular outflow
velocity profile left ventricle (LV) outflow. The index of combined
left ventricular systolic and diastolic function (the sum of isovolumic contraction time and isovolumic relaxation time divided by
ejection time) is calculated as (a b)/b.
TWO-DIMENSIONAL SPECKLE
TRACKING ECHOCARDIOGRAPHY
AND VELOCITY VECTOR IMAGING
In addition to inward and longitudinal motion, the LV also
rotates and twists during the cardiac cycle. To quantify the
complexity of cardiac motion, a technique called speckle
tracking has been developed. Speckles are small groups
of myocardial pixels created by the interaction between
ultrasound beams and the myocardium.11 Many vendors
have developed algorithms for tracking these speckles.
Speckle tracking measures aspects of strain, or myocardial
deformation, that occur during the cardiac cycle. Radial
strain (thickening of the myocardium during the inward
motion of the ventricle), longitudinal strain (percentage
decrease in length of the myocardium during systole as the
base moves toward the apex), and circumferential strain
(change in length along the circumferential perimeter) can
be assessed.11 In addition to measuring strain and strain
rate, speckle tracking also assesses the rotation, twist, and
Chapter 51: M-Mode and Two-Dimensional Echocardiographic Assessment of Left Ventricular Systolic Function
1121
Figs 51.8A and B: Contrast echocardiography. (A) Precontrast. The left ventricle (LV) cavity shows multiple trabeculations
(arrowhead) in the apex consistent with noncompaction. LV endocardial border is not well visualized in this area; (B) Postcontrast. Following injection of the contrast agent, the LV cavity is completely filled with contrast echoes, resulting in complete delineation of the
endocardium.
CONTRAST ECHOCARDIOGRAPHY
IN THE ASSESSMENT OF LEFT
VENTRICULAR SYSTOLIC FUNCTION
Commercially available echo contrast agents are widely
used to assist in determination of left ventricular systolic
function and evaluation of cardiac chambers and
myocardial perfusion. In patients with poor acoustic
windows, contrast can be given to help enhance detection
of the endocardial border and result in more accurate
visual estimates of left ventricular systolic function and
measurements of left ventricular volumes. Echo contrast
agents consist of reflective microbubbles. They are injected
intravenously and pass through right heart, the pulmonary
circulation, and into the left side of the heart, where they
opacify the left heart chambers and help delineate the
endocardial borders. The technique is similar to the more
invasive left ventriculogram obtained by injection of
contrast during left heart catheterization15,16 (Figs 51.8A
and B). Other features that can be adjusted to improve
contrast opacification include harmonic imaging and low
mechanical index imaging. With these advances, contrast
echocardiography provides improved endocardial border
imaging, resulting in better detection of wall motion
abnormalities, ventricular volume, and ejection fraction.
The result is a more accurate estimate of LV systolic
function.
ARTERIALVENTRICULAR COUPLING
The concept of arterialventricular coupling (EA/ELV) looks
at how properties of the arterial system affect the function
of the LV. Several studies have looked at how effective
arterial elastance (EA, arterial load) and left ventricular
end-systolic elastance (ELV, LV performance) relate and
affect cardiac performance especially in conditions
where the arterial tree becomes thicker and stiffer like
aging, heart failure, and hypertension.17 EA is calculated
as end-systolic pressure (ESP) divided by stroke volume
(SV) and serves as an index of the vascular load on the
LV. ESP is estimated as systolic blood pressure times 0.9
and SV is EDV ESV as obtained from 2D/Doppler echo
methods. ELV is noninvasively calculated using a modified
single-beat method to estimate end-systolic elastance
from arm-cuff pressures (systolic and diastolic BP), echoDoppler SV, echo-derived ejection fraction, and estimated
normalized ventricular elastance at arterial end-diastole.
It represents a relatively load-independent measure of LV
performance. Arterialventricular coupling is evaluated as
the ratio of these values (EA/ELV) and maximal efficiency is
attained when EA/ELV approaches 0.5.17 In states of elevated
afterload (e.g. aging, heart failure, and hypertension), there
is increased total peripheral resistance, left ventricular
concentric remodeling, inefficient arterialventricular
coupling, and ultimately, impaired LV function.18,19
1122
THREE-DIMENSIONAL TRANS
THORACIC ECHOCARDIOGRAPHY
With 2D transthoracic echocardiography (2D TTE), only
one slice of the LV can be obtained at a time. Obtaining
other slices to fully examine the LV requires moving the
transducer and adjusting the angle of the transducer in
various positions. With three-dimensional transthoracic
echocardiography (3D TTE), the transducer emits
hundreds of ultrasound waves through the heart allowing
one to obtain a full volume 3D data set of the entire LV.16 This
3D volume can then be cropped using any desired plane
angulation. For example, a single apically acquired 3D
data set potentially allows for display and analysis of all the
standard apical 2D views (apical 2-, 3-, 4-, and 5-chamber
views). This data set can also be used for analysis of shortaxis views from the apex to the base of the LV. In 2D echo,
many geometric assumptions are made about LV shape.16
As mentioned earlier, many of the formulas, including
the commonly used Simpsons biplane method of discs,
calculate LV volumes based on areas determined from
only two imaging planes.
According to a meta-analysis by Dorosz et al. Threedimensional echocardiography (3DE) provided more
precise and accurate quantification of LV volumes and
LVEF compared to two-dimensional echocardiography
(2DE).22 3DE volumes were obtained by either a slice
method or a mesh method. The slice method involved
manual tracing of equally spaced individual long-or shortaxis slices at end-systole and end-diastole. The mesh
REFERENCES
1. Pereztol-Valds O, Candell-Riera J, Santana-Boado C, et
al. Correspondence between left ventricular 17 myocardial
segments and coronary arteries. Eur Heart J. 2005;
26(24):263743.
2. Lang RM, Bierig M, Devereux RB, et al; Chamber
Quantification Writing Group; American Society of
Echocardiographys Guidelines and Standards Committee;
European Association of Echocardiography. Recomm
endations for chamber quantification: a report from the
American Society of Echocardiographys Guidelines and
Standards Committee and the Chamber Quantification
Writing Group, developed in conjunction with the
European Association of Echocardiography, a branch of
the European Society of Cardiology. J Am Soc Echocardiogr.
2005;18(12):144063.
3. Wilson DJ, North N, Wilson RA. Comparison of Left
Ventricular Ejection Fraction Calculation Methods.
Echocardiography. 1998;15(8 Pt 1):70912.
4. Feigenbaum, H. Role of M-mode technique in todays
echocardiography. J Am Soc Echocardiogr. 2010;23:24057.
5. Quinones MA, Waggoner AD, Reduto LA, et al. A new,
simplified and accurate method for determining ejection
fraction with two-dimensional echocardiography. Circu
lation. 1981;64(4):74453.
6. Baran AO, Rogal GJ, Nanda NC. Ejection fraction deter
mination without planimetry by two-dimensional
echocardiography: a new method. J Am Coll Cardiol.
1983;1(6):14718.
Chapter 51: M-Mode and Two-Dimensional Echocardiographic Assessment of Left Ventricular Systolic Function
1123
CHAPTER 52
How to Assess Diastolic Function
Hisham Dokainish
Snapshot
INTRODUCTION
In patients presenting with dyspnea, accurate assessment
of left ventricular (LV) systolic and diastolic function is of
utmost importance to establish or exclude heart failure as
a cause or component of dyspnea. Echocardiography with
Doppler readily assesses LV diastolic function; advantages
include that echocardiography is noninvasive, does not
require radiation, is portable, rapid, readily available,
and in competent hands, it can provide an accurate and
comprehensive assessment of LV systolic and diastolic
function. Correct assessment of LV diastolic function is
relevant in patients with both depressed and preserved LV
ejection fraction (EF < 50%, and 50%, respectively). Tissue
Doppler (TD) imaging has been useful in demonstrating
impaired LV relaxation in the setting of preserved left
ventricular ejection fraction (LVEF), which, in the setting
of increased cardiac volume, can result in elevated LV
filling pressures and dyspnea due to diastolic heart failure.
TD imaging is not always critical in patients with depressed
LVEF, since such patients by definition have impaired LV
relaxation, and thus significant increases in volume will
result in increases in LV filling pressure due to impaired
LV compliance. Thus, in depressed LVEF, transmitral
flow velocities (E and A, and E/A) and deceleration
time, pulmonary venous Doppler, left atrial volume, and
pulmonary artery (PA) pressures suffice for the accurate
assessment of LV filling pressures. Overall, diastolic
assessment by echo Doppler can be readily achieved in
Two-Dimensional Echocardiography:
Left Ventricular Mass and Wall Motion,
and Left Atrial Size
According to current guidelines, the following three
criteria are needed for the diagnosis of diastolic heart
failure (DHF): clinical picture consistent with HF, demon
stration of preserved LVEF, and demonstration of diastolic
dysfunction.1 Clinically, diastolic dysfunction, secondary
to impaired LV relaxation and increased LV stiffness, is
usually demonstrated by echocardiography and Doppler.26
The best correlate of symptoms and survival in DHF is
elevation of left atrial (or left ventricular filling) pressure,
readily estimated using comprehensive echocardiography
with Doppler.1,2 Demonstration of preserved LVEF is
readily demonstrated with 2D echocardiography.7 It should
be noted that DHF is a term used relatively intercha
ngeably with HF with preserved LVEF and HF with
normal LVEF.
In DHF, LVEF is preserved 50%, yet left atrial
pressuressynonymous with LV filling pressures in
the absence of obstructive mitral valve (MV) disease
are elevated, causing increased pulmonary venous
1125
Fig. 52.2: Presence of left atrial dilation in the patient with left
ventricular diastolic dysfunction. The same patient as in
Figure 52.1 has severely dilated left atrium (LA) from chronic
elevation in LA pressures in the setting of left ventricular hypertrophy from chronic hypertension. Note that the LA does not appear
significantly dilated by anteroposterior diameter in Figure. 52.1; this
is the reason current guidelines recommend the measurement of LA
volume in the apical views. This patient had severe LA enlargement,
with an unindexed LA volume of 137 mL and an indexed volume of
72 mL/m2.
1126
Transmitral Doppler
Pulsed Doppler interrogation of mitral valve diastolic
flow (mitral inflow pattern) is critical for the assessment
of LV filling pressures. Early mitral filling depends on
intrinsic LV relaxation, and the difference between LA and
LV early (or opening) diastolic pressure.5 In a healthy,
young heart with normal, rapid diastolic suction, the LV
literally sucks blood into the LV, resulting in rapid LA
emptying. In this scenario, there is a relatively tall E-wave
and a shorter A (late diastolic or atrial contraction wave;
Fig. 52.3). In an LV with impaired relaxation but normal
LV filling pressures, there is no rapid LV diastolic suction,
thus LA emptying is more gradual and results in a relatively
low velocity E-wave; LA emptying is therefore dependent
on LA contraction and results in a relatively high amplitude
A-wave. In the setting of impaired LV relaxation and mildly
elevated LA pressure, high LA pressure that drives open
the MV, resulting in a large E-wave and smaller A-wave;
Valsalva Maneuver
In the Valsalva maneuver in which the patient forces
expiration against a closed glottis, there is increased
intrathoracic pressure that results in decrease in right
heart filling which by definition, results in decreased LV
filling (decreased preload). Since a pseudonormal filling
pattern exists in the setting of elevated LA pressure in
the presence of impaired LV relaxation, this decrease in
preload lowers LA pressure, which then unmasks the
underlying impaired relaxation pattern (i.e. E > A in the
setting of impaired relaxation and with Valsalva maneuver
changes the transmitral pattern to E < A; Fig. 52.5). On the
1127
1128
Figs 52.6A to D: Tissue Doppler imaging for the assessment of left ventricular (LV) diastolic function. Figure A shows an apical fourchamber view with moderate to severe dilation of the left atrium (LA) with an LA volume index of 39 mL/m2; Figure B demonstrates
elevated early transmitral diastolic velocity (E) = 117 cm/s; Figure C shows very depressed mitral lateral annular early diastolic relaxation
velocity (e') = 3.5 cm/s; Figure D shows very depressed septal tissue Doppler (TD) early diastolic velocity (e') = 2.5 cm/s. Therefore,
E/e' septal = 47, and E/e' lateral = 33, indicating severely impaired LV relaxation with elevated LV filling pressures. (PCWP: Pulmonary
capillary wedge pressure).
1129
Fig. 52.8: Pulmonary venous Doppler velocities in the assessment of left atrial pressures. In normal heart, systolic pulmonary
venous (PV) flow (S) is lower than diastolic PV flow (D), as rapid
left ventricular (LV) suction during diastole results in elevated PV D
velocities; thus, PV S < D. In patients with impaired LV relaxation
and elevated LA pressure, when the mitral valve (MV) is closed in
ventricular systole, the elevated LA pressure prevents PV S flow,
and therefore most flow occurs when the MV opens, resulting in
S < D. Therefore, as with mitral inflow velocities, PV velocities
are also prone to pseudonormal filling. In this example, there is
impaired LV relaxation due to cardiomyopathy, but LA pressure
is not elevated; thus, when the MV is closed (LV systole), there is
unimpeded flow through the PV, and hence S > D.
1130
Figs 52.9A and B: Pulmonary artery systolic pressure in the assessment of left ventricular diastolic function. In patients with significant left ventricular (LV) diastolic dysfunction with chronically elevated LV filling pressures, back pressure through the left atrium (LA),
into the pulmonary veins, and across the pulmonary venous capillary bed into the pulmonary arterioles and pulmonary arteries (PA),
results in elevation of PA pressure. Thus, PA systolic pressure elevation, in the absence of significant intrinsic lung disease and resultant
elevated pulmonary vascular resistance (PVR) is a reasonable correlate of elevated LA pressures. PA systolic pressure can be estimated by Doppler using the tricuspid regurgitation peak systolic velocity and adding to it an estimate of right atrial (RA) pressure. This image
shows a TR velocity of 3.64 m/s, equivalent to a TR systolic pressure of 53 mm Hg, which indicates at least moderate PA hypertension
in a patient with chronically elevated LA pressure due to ischemic cardiomyopathy and diastolic dysfunction. (RV: Right ventricular).
Fig. 52.10: An integrated approach to the assessment of left ventricular diastolic function: normal LV ejection fraction. As recommended in the current guidelines, use of multiple echo Doppler
parameters results in a more accurate assessment of left ventricular (LV) diastolic function than using any single echo Doppler
parameter in isolation. In the patient with normal LV ejection fraction (EF), it is reasonable to start with early transmitral diastolic
velocity/tissue Doppler early diastolic velocity (E/e'), as it can be
difficult to discern whether a patient with preserved LVEF has impaired or normal LV relaxation. Following E/e', other echo Doppler
variables are added to result in an accurate assessment of LV
diastolic function (from ref. 5).
INTEGRATING ECHOCARDIOGRAPHIC
VARIABLES FOR ACCURATE
DIAGNOSIS OF DIASTOLIC FUNCTION
The use of a single diastolic variable (such as E/e' or LA
volume in isolation) can lead to significant errors in
the assessment of LV diastolic function.5 It is therefore
1131
1132
SUMMARY
Comprehensive echocardiography with 2D imaging, and
spectral and color Doppleras well as newer techniques
like speckle strain echocardiographyprovide a complete
assessment of cardiac diastolic function. This assessment,
which includes LV mass and regional wall motion
assessment, LA volume, transmitral, pulmonary venous,
and tissue Doppler as well as estimation of PA systolic and
diastolic pressures, can provide accurate assessments of
diastolic function in the majority of patients. It is important
to note that, as recommended in current guidelines, use
of any single echo Doppler diastolic variable (e.g. only
E/e') in isolation, can lead to errors. Therefore, it is of
utmost importance that a comprehensive assessment of
LV diastolic function include integration of all available 2D
and Doppler, and tissue Doppler variables to arrive at the
most accurate diastolic assessment.
REFERENCES
1. Paulus WJ, Tschpe C, Sanderson JE, et al. How to diagnose
diastolic heart failure: a consensus statement on the diag
nosis of heart failure with normal left ventricular ejection
fraction by the Heart Failure and Echocardiography
Associations of the European Society of Cardiology. Eur
Heart J. 2007;28(20):253950.
2. Zile MR, Baicu CF, Gaasch WH. Diastolic heart failure
abnormalities in active relaxation and passive stiffness of
the left ventricle. N Engl J Med. 2004;350(19):19539.
3. Oh JK, Hatle L, Tajik AJ, et al. Diastolic heart failure can be
diagnosed by comprehensive two-dimensional and Doppler
echocardiography. J Am Coll Cardiol. 2006;47(3):5006.
17. Nagueh SF, Lakkis NM, Middleton KJ, et al. Doppler
estimation of left ventricular filling pressures in patients
with hypertrophic cardiomyopathy. Circulation. 1999;99(2):
25461.
18. Nagueh SF, Mikati I, Kopelen HA, et al. Doppler estimation
of left ventricular filling pressure in sinus tachycardia. A
new application of tissue Doppler imaging. Circulation.
1998;98(16):164450.
19. Sohn DW, Song JM, Zo JH, et al. Mitral annulus velocity in
the evaluation of left ventricular diastolic function in atrial
fibrillation. J Am Soc Echocardiogr. 1999;12(11):92731.
20. Sundereswaran L, Nagueh SF, Vardan S, et al. Estimation
of left and right ventricular filling pressures after heart
transplantation by tissue Doppler imaging. Am J Cardiol.
1998;82(3):3527.
21. Yu CM, Sanderson JE, Marwick TH, et al. Tissue Doppler
imaging a new prognosticator for cardiovascular diseases.
J Am Coll Cardiol. 2007;49(19):190314.
22. Garcia MJ, Ares MA, Asher C, et al. An index of early left
ventricular filling that combined with pulsed Doppler peak
E velocity may estimate capillary wedge pressure. J Am Coll
Cardiol. 1997;29(2):44854.
23. Rivas-Gotz C, Manolios M, Thohan V, et al. Impact of left
ventricular ejection fraction on estimation of left ventri
cular filling pressures using tissue Doppler and flow
propagation velocity. Am J Cardiol. 2003;91(6):7804.
24. Appleton CP, Galloway JM, Gonzalez MS, et al. Estimation
of left ventricular filling pressures using two-dimensional
and Doppler echocardiography in adult patients with
cardiac disease. Additional value of analyzing left atrial
size, left atrial ejection fraction and the difference in
duration of pulmonary venous and mitral flow velocity at
atrial contraction. J Am Coll Cardiol. 1993;22(7):197282.
25. Neuman Y, Kotliroff A, Bental T, et al. Pulmonary artery
pressure and diastolic dysfunction in normal left ventricular
systolic function. Int J Cardiol. 2008;127(2):1748.
26. Paraskevaidis IA, Tsiapras DP, Karavolias GK, et al. Dopplerderived left ventricular end-diastolic pressure prediction
model using the combined analysis of mitral and
pulmonary A waves in patients with coronary artery
disease and preserved left ventricular systolic function.
Am J Cardiol. 2002;90(7):7204.
1133
CHAPTER 53
Evaluation of the Right Ventricle
Vincent L Sorrell, Steve W Leung, Brandon Fornwalt
Snapshot
General Overview
Echocardiography
M-Mode Echocardiography
Two-Dimensional Echocardiography
Doppler Echocardiography
GENERAL OVERVIEW
The assessment of the right ventricle (RV) is valuable in
many patients with heart disease. In patients with either
RV volume overload (e.g. repaired tetralogy of Fallot [TOF],
atrial septal defect [ASD], anomalous pulmonary venous
return, tricuspid regurgitation [TR] from any cause) or
RV pressure overload (e.g. pulmonary hypertension from
any cause, pulmonary stenosis), management decisions
increasingly rely on evaluation of the RV size and function.
Their trends during serial follow-up examinations
predict heart failure, arrhythmias, and death and must
be reliable.1 The noninvasive diagnostic evaluation of RV
size and function in normal and pathological conditions
is daunting due to its complex shape, nonsymmetrical
regional contraction pattern, and the lack of published
literature on normal reference values. Most physicians
practicing echocardiography are comfortable analyzing
the relatively simple circular geometry of the left ventricle
(LV), but the RV is shaped like a pyramidal banana. The
inflow and outflow portions are separated. The normal RV
shape varies depending on orientation: sagittal view (echo
Three-Dimensional Echocardiography
Transesophageal Echocardiography
Hemodynamics
1135
1136
ECHOCARDIOGRAPHY
Fig. 53.3: Cardiac magnetic resonance imaging midventricular
steady-state free precession (SSFP) images of a normal (control;
top) and pathological (tetralogy; bottom) heart demonstrating the
variation in timevolume (TV) curves (right side). Note the dilated
right ventricle (RV) and delay in contraction of the surgically repaired tetralogy of Fallot patient (tetralogy). Right ventricular endocardial tracing and TV curves, green; left ventricular endocardial tracing and TV curves, yellow.
M-MODE ECHOCARDIOGRAPHY
The myofibrillar arrangement of the RV consists of mainly
subepicardial circumferential fibers and subendocardial
longitudinal fibers in the inflow region and both
subepicardial and subendocardial longitudinal fibers in
the outflow region. The majority of the RV myocardium
1137
Fig. 53.4: Echo windows for right ventricle (RV) assessment. Graphical illustration of the 11 recommended two-dimensional (2D) echocardiographic images from a transthoracic approach highlighting the parasternal long, parasternal short, apical, and subcostal views to
obtain a comprehensive assessment of RV size and function. For additional details, see Rudski LG, et al. Guidelines for the Echocardiographic Assessment of the Right Heart in Adults: A Report from the American Society of Echocardiography. J Am Soc Echocardiogr.
2010;23:685713.
1138
Fig. 53.5: Two different patients two-dimensional (2D) echocardiograms, apical four-chamber orientation (top row), diastolic (left)
and systolic (right) frames, and associated M-mode from the right
ventricle (RV) tricuspid annulus (bottom row). The patient displayed on the left has a normal RV and the patient displayed on
the right has marked RV dilation and dysfunction. Arrows represent the systolic excursion of the tricuspid annular plane systolic
excursion (TAPSE).
TWO-DIMENSIONAL
ECHOCARDIOGRAPHY
The RV is typically smaller than the LV when normal, and
normally viewed in the apical four-chamber view using
2D echo (2DE). However, it may be difficult to confirm
the optimal alignment of these ventricles. Therefore, using
relative dimensions as the sole criterion to diagnose RV
Fig. 53.7: Two-dimensional (2D) echocardiogram, short-axis orientation, midventricular position (left), and accompanying schematic
(inset). Images on the right represent the apical four-chamber
cut-planes and accompanying schematic (inset). When aligned
correctly through the midcavity of the left ventricle (LV: Solid white
line), the LV/right ventricle (RV) ratio is > 1.5:1. When aligned
incorrectly superior (dashed yellow line) or inferior (dashed pink
line), this normal ratio may change and the normal RV may inadvertently appear relatively dilated. Although these lines are graphically displayed as parallel, in actual clinical practice these arise
from the same transducer location point and are more divergent.
1139
Fig. 53.8: See Movie clip 53.6A. Contrast-enhanced two-dimensional echocardiogram, zoom apical four-chamber orientation,
focused on the right ventricle (RV) apex. The center dark line represents the ventricular septum (myocardium). The unenhanced,
well-circumscribed, 2.0 cm 1.5 cm filling defect in the RV apex
represents a large RV thrombus. The dark region toward the base
of the RV cavity represents attenuation artifact from the dense
manufactured contrast agent.
DOPPLER ECHOCARDIOGRAPHY
Conventional Doppler
Importantly, all conventional Doppler techniques are
subject to increased error as the quality of the spectral
Doppler signal worsens and therefore, special care
should be taken during image acquisition. The rate of RV
pressure increase is derived from the continuous wave
Doppler (CWD) spectral display of the TR signal. The time
interval (dt) necessary to increase the TR velocity from
baseline to 2.0 m/s represents a change in pressure (dP) of
16 mm Hg. When the TR velocity is elevated, time intervals
from 1.0 m/s to 2.5 or 3.0 m/s (dP = 21 mm Hg and 32 mm
Hg, respectively) can alternatively be obtained (similar to
LV dP/dt estimates) and reduce error from using very low
velocities. The dP/dt value is considered normal when >400
mm Hg/s (Fig. 53.10).20 Since the dP/dt is dependent on
preload, the maximal TR velocity (TRmax) can be included
in the equation (dP/dt/TRmax) and partially compensate
for this.21 In patients with predominant RV failure, the TRderived dP/dt/TRmax, but not dP/dt alone, was shown to
be a clinically useful index of global RV contractility.
1140
Fig. 53.9: Two-dimensional (2D) echocardiogram, apical fourchamber orientation, diastolic (left) and systolic frame (right).
The right ventricle (RV) endocardial border has been traced for
estimating the volumes (and calculating the fractional area change
[FAC]) using the Simpsons method of discs. In this patient with
severe global RV systolic dysfunction, the FAC was 13%. (FAC:
Fractional area change; RVd: RV diastolic volume; RVs: RV
systolic volume).
Tissue Doppler
The pulmonary circulation normally has a low vascular
resistance, and consequently, a very short (or unde
tectable) isovolumic contraction time (IVCT) and isovo
lumic relaxation time (IVRT). The superficial circum
ferential fibers contract during the IVCT and the deeper
longitudinal fibers contract during ejection. The onset of
RV ejection at the outflow tract is delayed after the onset of
contraction of the inflow tract. This regional RV contractility
requires high temporal resolution to be recognized and
provides a basis for color mapping of the myocardium with
advanced tissue Doppler or speckle tracking techniques.
1141
Fig. 53.11: Two-dimensional (2D) echocardiogram, apical fourchamber orientation, tissue Doppler color map display of mean
myocardial displacement (derivative of velocity/time). The arrow
points to the basal right ventricle (RV) myocardial region, which
we have found should remain purple ( 12 mm) for approximately
50% of the length of the RV free wall in normal individuals. This
is a quick semiquantitative tool that appears to correlate with
tricuspid annular plane systolic excursion (TAPSE).
TWO-DIMENSIONAL STRAIN
(SPECKLE TRACKING)
The RV myocardium is normally only 34
Within this thin layer of myocardium resides
arrangement of circumferential (parallel to
valve (AV) groove and encircling the RVOT)
mm thin.
a complex
the aortic
and spiral
1142
Figs 53.14A and B: Two-dimensional (2D) echocardiogram, apical four-chamber orientation, angulated slightly rightward to visualize
the entire right ventricle (RV) apex with 2D strain color map (speckle tracking) display of the RV myocardium. A. Diastolic frame (arrow =
apical variant hypertrophic cardiomyopathy); B. Systolic frame (arrow = abnormal distal ventricular septum due to adjacent pathological
LV myocardium). For additional details, see Abdy NA, et al. Apical Hypertrophic Cardiomyopathy in an Adolescent. Congen Heart Dis.
2010;5(2):18287.
THREE-DIMENSIONAL
ECHOCARDIOGRAPHY
In the absence of cardiac shunting (or significant atrioven
tricular valve regurgitation), the LV and RV have the same
1143
Volume
CMR (mL/m2)
RT3DE (mL/m2)
Mean (SD)
Mean (SD)
EDV:
All
71
71.3 (12.9)
70.0 (12.9)
Male
36
67.1 (12.1)
56.4 (13.4)
Female
35
75.6 (12.4)
74.7 (13.0)
All
71
33.5 (9.9)
33.4 (10.3)
Male
36
28.6 (8.1)
29.2 (10.7)
35
38.4 (9.1)
37.8 (7.4)
ESV:
Female
RVEF:
All
71
53.3 (8.7)
52.6 (9.9)
Male
36
57.5 (7.0)
56.2 (9.1)
Female
35
49.0 (8.8)
48.9 (9.5)
Normal right ventricular volumes (indexed to body surface area) as measured with CMR and RT3DE categorized by gender to enddiastolic and end-systolic volumes and the resulting RVEF. Table modified from Reference 38. For additional details, see Gopal AS,
et al. Normal values of right ventricular size and function by real time three-dimensional echocardiography: comparison to cardiac
magnetic resonance imaging. J Am Soc Echocardiogr. 2007;20:44555.
(CMR: Cardiac magnetic resonance imaging; EDV: End-diastolic volume; ESV: End-systolic volume; RT3DE: Real time three-dimensinal echocardiography; RVEF: Right ventricular ejection fraction).
TRANSESOPHAGEAL
ECHOCARDIOGRAPHY
When the transthoracic ultrasound window is suboptimal
and CMR is not available, transesophageal echo (TEE)
may be performed. Most reports on TEE evaluation of
the RV are from intraoperative studies and may not be
as clinically relevant due to the frequent administration
of inotropic medications and rapid fluid shifts in this
population. In a study of 25 children operated on for ASDs,
90% had adequate 3D TEE studies.40 RV volumes with this
HEMODYNAMICS
Although not a direct estimate of RV volume or function,
the evaluation of right heart hemodynamics is exceedingly
valuable when right heart pathology is suspected. The
right atrial pressure (RAP) is readily estimated from
the inferior vena cava (IVC) dynamics and the caval
and HV flows. More recently, DTI has also been used to
evaluate this parameter. Classically, a dilated IVC, lack of
inspiratory collapse, E/e' ratio > 6, atrial septal leftward
bulge, predominant diastolic flow patterns in the SVC,
or HVs suggest an elevated RAP. However, these features
may be normal in athletes, obese individuals, congenital
narrowing of the IVCRA junction (BuddChiari), cor
triatriatum dexter, or mechanical ventilation. Importantly,
IVC imaging should be performed in the supine (not left
lateral decubitus) position. For specific RAP values, it is
somewhat reliable to use 020 mm Hg range at 5 mm Hg
intervals. If the IVC size is < 21 mm and the inspiratory
1144
Fig. 53.16: See Movie clip 53.10. Cardiac CTA displayed in the
four-chamber orientation demonstrating the clearly defined border
between the brighter blood pool and the darker right ventricular
myocardium.
1145
CONCLUSION
Continued investigation is warranted to improve our
understanding of the RV and to obtain robust noninvasive
diagnostic methods to assess this chamber, which
continues to be proven to predict clinical outcomes.
Evolving clinical settings demonstrate the importance of
evaluating the RV in an effort to predict RV failure such as
prior to left ventricular assist devices. Given the normally
complex shape, the further distortion with pathology, and
the intricate myofibrillar arrangement, it is likely that a
combination of parameters (possibly imaging and clinical)
will need to be used for optimal RV assessment rather than
a single diagnostic parameter. It may also be necessary to
combine diagnostic imaging tools, but for the foreseeable
future, echo will be the initial technique for this purpose.
DISCLOSURE
Gadolinium contrast is not Food and Drug Administration
(FDA) approved for CMR.
REFERENCES
1. Oosterhof T, van Straten A, Vliegen HW, et al. Preoperative
thresholds for pulmonary valve replacement in patients
with corrected tetralogy of Fallot using cardiovascular
magnetic resonance. Circulation. 2007;116(5):54551.
1146
31. Schwerzmann M, Samman AM, Salehian O, et al.
Comparison of echocardiographic and cardiac magnetic
resonance imaging for assessing right ventricular function
in adults with repaired tetralogy of fallot. Am J Cardiol.
2007;99(11):15937.
32. Pirat B, McCulloch ML, Zoghbi WA. Evaluation of global
and regional right ventricular systolic function in patients
with pulmonary hypertension using a novel speckle
tracking method. Am J Cardiol. 2006;98(5):699704.
33. Teske AJ, De Boeck BW, Olimulder M, et al. Echocar
diographic assessment of regional right ventricular function:
a head-to-head comparison between 2-dimensional and
tissue Doppler-derived strain analysis. J Am Soc Echo
cardiogr. 2008;21(3):27583.
34. Abdy NA, Valdes SO, Sorrell VL, et al. Apical hypertrophic
cardiomyopathy in an adolescent. Congenit Heart Dis.
2010;5(2):182187.
35. Weidemann F, Jamal F, Sutherland GR, et al. Myocardial
function defined by strain rate and strain during alterations
in inotropic states and heart rate. Am J Physiol Heart Circ
Physiol. 2002;283(2):H792H799.
36. Grant AD, Smedira NG, Starling RC, et al. Independent
and incremental role of quantitative right ventricular
evaluation for the prediction of right ventricular failure
after left ventricular assist device implantation. J Am Coll
Cardiol. 2012;60(6):5218.
37. Jenkins C, Chan J, Bricknell K, Strudwick M, Marwick
TH. Reproducibility of right ventricular volumes and
ejection fraction using real-time three-dimensional
echocardiography: comparison with cardiac MRI. Chest.
2007;131(6):184451.
38. Gopal AS, Chukwu EO, Iwuchukwu CJ, et al. Normal
values of right ventricular size and function by real-time
3-dimensional echocardiography: comparison with cardiac
magnetic resonance imaging. J Am Soc Echocardiogr.
2007;20(5):44555.
39. Shiota T. 3D echocardiography: evaluation of the right
ventricle. Curr Opin Cardiol. 2009;24(5):4104.
40. Grison A, Maschietto N, Reffo E, et al. Three-dimensional
echocardiographic evaluation of right ventricular volume
and function in pediatric patients: validation of the
technique. J Am Soc Echocardiogr. 2007;20(8):9219.
41. Kircher BJ, Himelman RB, Schiller NB. Noninvasive
estimation of right atrial pressure from the inspiratory
collapse of the inferior vena cava. Am J Cardiol.
1990;66(4):4936.
42. Nagueh SF, Kopelen HA, Zoghbi WA. Relation of mean
right atrial pressure to echocardiographic and Doppler
parameters of right atrial and right ventricular function.
Circulation. 1996;93(6):11609.
43. Sade LE, Gulmez O, Eroglu S, et al. Noninvasive estimation
of right ventricular filling pressure by ratio of early tricuspid
inflow to annular diastolic velocity in patients with and
without recent cardiac surgery. J Am Soc Echocardiogr.
2007;20(8):9828.
1147
44. Sorrell VL, Reeves WC. Noninvasive right and left heart
catheterization: taking the echo lab beyond an image-only
laboratory. Echocardiography. 2001;18(1):3141.
45. Selton-Suty C, Juillire Y. Non-invasive investigations
of the right heart: how and why? Arch Cardiovasc Dis.
2009;102(3):21932.
46. Sorrell VL, Indik JI, Marcus FI. Right ventricular cardio
myopathies. Section III. Chapter 19. Cardiovascular
multimodal imaging in key clinical problems. In: Pahlm
O, Wagner G, editors. Multimodal Cardiovascular Imaging:
Principles and Clinical Applications. McGraw-Hill; 2011.
47. Grothues F, Moon JC, Bellenger NG, et al. Interstudy
reproducibility of right ventricular volumes, function, and
mass with cardiovascular magnetic resonance. Am Heart
J. 2004;147(2):21823.
48. Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis
of arrhythmogenic right ventricular cardiomyopathy/
dysplasia: proposed modification of the task force criteria.
Circulation. 2010;121(13):153341.
49. Bradlow WM, Gibbs JS, Mohiaddin RH. Cardiovascular
magnetic resonance in pulmonary hypertension.
J Cardiovasc Magn Reson. 2012;14:6.
50. Warnes CA, Williams RG, Bashore TM, et al.; American
College of Cardiology; American Heart Association Task
Force on Practice Guidelines (Writing Committee to Develop
Guidelines on the Management of Adults with Congenital
Heart Disease); American Society of Echo
cardiography;
Heart Rhythm Society; International Society for Adult
Congenital Heart Disease; Society for Cardiovascular
Angiography and Interventions; Society of Thoracic
Surgeons. ACC/AHA 2008 guidelines for the management
of adults with congenital heart disease: a report of the
American College of Cardiology/American Heart Association
Task Force on Practice Guidelines (Writing Committee to
Develop Guidelines on the Management of Adults with
Congenital Heart Disease). Developed in Collaboration
with the American Society of Echocardiography, Heart
Rhythm Society, International Society for Adult Congenital
Heart Disease, Society for Cardiovascular Angiography and
Interventions, and Society of Thoracic Surgeons. J Am Coll
Cardiol. 2008;52(23):e143e263.
51. Sorrell VL, Altbach MI, Kudithipudi V, et al. Cardiac MRI
is an important complementary tool to Doppler echocar
diography in the management of patients with pulmonary
regurgitation. Echocardiography. 2007;24(3): 31628.
52. Kumar A, Abdel-Aty H, Kriedemann I, et al. Contrastenhanced cardiovascular magnetic resonance imaging of
right ventricular infarction. J Am Coll Cardiol. 2006;48(10):
196976.
53. Lockie T, Nagel E, Redwood S, et al. Use of cardiovascular
magnetic resonance imaging in acute coronary syndromes.
Circulation. 2009;119(12):167181.
54. Weinsaft JW, Kim RJ, Ross M, et al. Contrast-enhanced
anatomic imaging as compared to contrast-enhanced
tissue characterization for detection of left ventricular
thrombus. JACC Cardiovasc Imaging. 2009;2(8):96979.
1148
CHAPTER 54
Three-Dimensional Echocardiographic
Assessment of LV and RV Function
Aasha S Gopal
Snapshot
INTRODUCTION
Significant progress has taken place in the last 25 years
in moving echocardiography from a two-dimensional
(2D) imaging modality to a three-dimensional (3D)
imaging modality that has found several routine clinical
applications, an important one being quantification of
cardiac structure and function. This progress has closely
paralleled the transition in transducer technology from
conventional phased-array transducers to matrix array
transducers. This chapter explores the limitations of
quantifying left and right ventricular (RV) structure and
function by conventional M-mode [one-dimensional
(1D)] and 2D echocardiography (2DE), thereby providing
the rationale for developing and adopting new methods
such as 3D echocardiography (3DE) and speckle tracking
echocardiography (STE).
3D QUANTITATION OF THE
LEFT VENTRICLE
Limitations of 2D Echocardiography
Lack of 3D Spatial Coordinates
Left ventricular ejection fraction (LVEF) is a cardinal
parameter that has been shown to have tremendous
prognostic value in a variety of clinical situations varying
from valvular heart disease, ischemic heart disease
1150
Figs 54.2A to C: Freehand scanning. A modified ultrasound probe is tracked in three-dimensional (3D) space using an electromagnetic
field device; images then may be reconstructed offline to create 3D data sets. A schematic of the receiver and transmitting device and
the Cartesian coordinate system for tracking the location of the transducer is shown. (Courtesy of TomTec Imaging Systems, Munich,
Germany; with permission).
Limitations of 2D Echocardiography
Geometric Assumptions
Since conventional echocardiography does not provide
cross-sectional images that are spatially related to each
other in a precise fashion, it is necessary to make certain
assumptions about ventricular geometry to arrive at a
more objective assessment of LV size and function. Linear
measurements are popular and still reported today.
However, they are difficult to reproduce when acquired
in an unguided fashion and they assume that ventricular
enlargement occurs uniformly and is reflected faithfully in
the increase in the linear dimension. For example, when
1151
Fig. 54.3: Schematic of how three-dimensional (3D) transesophageal echo (TEE) was acquired. The TEE probe is shown with its range
of rotation from 180. Typically, a two-dimensional (2D) image was acquired every 3. The relationship between the heart and the TEE
rotation is shown. (Courtesy of TomTec Imaging Systems, Munch, Germany; with permission.)
1152
Limitations of 2D Echocardiography
Apical Foreshortening and Boundary
Recognition
2DE from the transthoracic approach has additional
limitations that are patient specific and inherent to the
anatomy and position of the heart as it is situated in the
rib cage. Imaging is performed through the interspaces
between the ribs and even though guided 3DE can achieve
perfect image positioning, image quality may be degraded if
there is no optimal transthoracic interspace. Inherent ways
of compensating for the lack of an adequate rib interspace is
to utilize whatever echocardiographic window is available.
This can frequently lead to apical foreshortening as was
nicely demonstrated in a simultaneous echocardiographic
and cineventriculographic study.21 Similarly, it is possible
to obtain only tangential cross-sections of the heart that
may cause inaccuracies in estimating linear dimensions
and wall thickness.
Limitations of 3D Reconstruction
Although 3D reconstruction addresses the principal
limitations to accurate quantitation of the left ventricle,
namely image position error and geometric assumptions,
this methodology has several limitations. The images
acquired for 3D reconstruction are nonsimultaneous.
Therefore, it may be inaccurate in patients with significant
intracardiac dyssynchrony. It also requires electrocardiogram (ECG) gating and is susceptible to inaccuracies due
1153
Figs 54.6A and B: (A) First-generation matrix array transducer: Simultaneous display of parasternal long-axis (orthogonal B scan) and
short-axis views (C-scan); (B) First-generation matrix array transducer: Simultaneous display of apical (orthogonal B scan) and shortaxis views (C-scan). (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
1154
Figs 54.7A and B: (A) Phased-array elements compared to the size of a human hair; (B) Matrix array elements compared to the size
of a human hair.
1155
Figs 54.10A and B: (A) Full-volume three-dimensional (3D) data set acquired by a third-generation matrix array transducer. The data
set is displayed as a set of three image planes that are spaced 60 apart and one cross-sectional axial image; (B) Three equiangular
planes (triplane) that are spaced 60 apart can be generated selectively instead of acquiring a full-volume data set and used for calculation of 3D volumes and ejection fraction. The image quality of triplane imaging is superior to that of full-volume imaging and therefore,
may have some advantages for left ventricular quantitation.
1156
1157
1158
Fig. 54.11: Peak filling rate (PFR) derived from RT 3DE time volume
curves fitted to third order harmonics normal response-rest and
stress. (RT 3DE: Real time, three-dimensional echocardiography).
Fig. 54.12: Peak filling rate (PFR) derived from RT 3DE time volume
curves fitted to third order harmonics-ischemic response-rest and
stress. (RT 3DE: Real time, three-dimensional echocardiography).
1159
Myocardial ischemia
Stress-rest differences
PFR > 0
PFR < 0
SDS < 2
13
SDS > 2
1160
Fig. 54.16: Strain in two dimensions. Above are the two normal
strains along the x and y axes, where each strain component can
be seen as Lagrangian strain along one main axis. Below are the
two shear strain components, movement of the borders relative to
each other. Here there are two strain components, characterized
by the tangent to the shear angle alpha.
Source: Reproduced with permission from http://folk.ntnu.no/
stoylen/strainrate.
Fig. 54.17: Strain in three dimensions. Only the three strain components along the x axis (one normal, two shear) are shown, but the
y and z strains will be exactly the same and can be imagined by rotating the x images.
Source: Reproduced with permission from: http://folk.ntnu.no/stoylen/strainrate.
1161
1162
Fig. 54.20: The left ventricle (LV) has been subdivided into 17
regional segments. The timevolume curve for each segment is
displayed. In this normal subject without significant intracardiac
dyssynchrony, the minima of the timevolume curves (shown on
the top panel) and their first derivatives (bottom panel) are all
reached at roughly the same time.
Fig. 54.21: The left ventricle has been subdivided into 17 regional
segments. The time-volume curve for each segment is displayed.
In this patient with significant intracardiac dyssynchrony, the minima of the timevolume curves (shown on the top panel) and their
first derivatives (bottom panel) have a wide temporal dispersion.
1163
1164
1165
Table 54.2: Misclassification rate of EF postmyocardial infarction (comparison of clinical EF, 2DE EF, and 3DE EF to CMREF)
25%
CMR EF = 40%
CMR EF = 30%
Clinical EF
42.4%
15.2%
2DE EF
21.8%
10.9%
3DE EF
14.5%
5.4%
1166
1167
1168
1169
Fig. 54.34: RT 3DE-disc summation methodthe same threedimensional (3D) data set shown in Figure 54.4A is now advanced
to show three orthogonal MPRs (top left, top right and bottom left
panels) of the RV taken at the mid-ventricular level. (RT 3DE: Real
time, three-dimensional echocardiography; RV: Right ventricle).
1170
REFERENCES
1. Amico AF, Lichtenberg GS, Reisner SA, et al. Superiority of
visual versus computerized echocardiographic estimation
of radionuclide left ventricular ejection fraction. Am Heart J.
1989;118(6):125965.
2. King DL, Harrison MR, King DL Jr, et al. Ultrasound beam
orientation during standard two-dimensional imaging:
assessment by three-dimensional echocardiography. J Am
Soc Echocardiogr. 1992;5(6):56976.
3. King DL, King DL Jr, Shao MY. Three-dimensional spatial
registration and interactive display of position and orientation of real-time ultrasound images. J Ultrasound Med.
1990;9(9):52532.
4. Levine RA , Handschumacher MD, Sanfilippo AJ, et al.
Three-dimensional echocardiographic reconstruction of
the mitral valve, with implications for the diagnosis of
mitral valve prolapse. Circulation. 1989;80(3):58998.
5. Xu J, Wu Y, Li X, et al. Accuracy of three-dimensional
quantification of left ventricular function using magnetic
sensor acquisition: a dynamic in vitro model. Chin Med J.
2001;114(10):101114.
6. King DL, Harrison MR, King DL Jr, et al. Improved reproducibility of left atrial and left ventricular measurements
by guided three-dimensional echocardiography. J Am Coll
Cardiol. 1992;20(5):123845.
1171
1172
61. Byrd BF 3rd, Wahr D, Wang YS, et al. Left ventricular mass
and volume/mass ratio determined by two-dimensional
echocardiography in normal adults. J Am Coll Cardiol.
1985;6(5):10215.
62. Gottdiener JS, Livengood SV, Meyer PS, et al. Should echocardiography be performed to assess effects of antihypertensive therapy? Test-retest reliability of echocardiography
for measurement of left ventricular mass and function.
J Am Coll Cardiol. 1995;25(2):42430.
63. Sapin PM, Gopal AS, Clarke GB, et al. Three-dimensional
echocardiography compared to two-dimensional echocardiography for measurement of left ventricular mass
anatomic validation in an open chest canine model. Am
J Hypertens. 1996;9(5):46774.
64. Gopal AS, Keller AM, Shen Z, et al. Three-dimensional
echocardiography: in vitro and in vivo validation of left
ventricular mass and comparison with conventional echocardiographic methods. J Am Coll Cardiol. 1994;24(2):
50413.
65. Gopal AS, Schnellbaecher MJ, Shen Z, et al. Freehand
three-dimensional echocardiography for measurement of
left ventricular mass: in vivo anatomic validation using
explanted human hearts. J Am Coll Cardiol. 1997;30(3):
80210.
66. Mor-Avi V, Sugeng L, Weinert L, et al. Fast measurement
of left ventricular mass with real-time three-dimensional
echocardiography: comparison with magnetic resonance
imaging. Circulation. 2004;110(13):18148.
67. Chang SA, Kim HK, Lee SC, et al. Assessment of left
ventricular mass in hypertrophic cardiomyopathy by realtime three-dimensional echocardiography using single-beat
capture image. J Am Soc Echocardiogr. 2013;26(4):43642.
68. Shimada YJ, Shiota T. Meta-analysis of accuracy of left
ventricular mass measurement by three-dimensional
echocardiography. Am J Cardiol. 2012;110(3):44552.
69. King DL, King Jr. DL, Gopal AS, et al. Myocardial
infarct reconstruction and sizing by three-dimensional
echocardiography. In: Proceedings of the Computers in
Cardiology 1992; October 1114, 1992, Durham, NC. IEEE
Computer Society Press. Los Alamitos, CA. pp. 62730.
70. King DL, Gopal AS, King DL Jr, et al. Three-dimensional
echocardiography: in vitro validation for quantitative
measurement of total and infarct surface area. J Am Soc
Echocardiogr. 1993;6(1):6976.
71. Gopal AS, Keller AM, Rigling R, et al. Left ventricular
volume and endocardial surface area by three-dimensional
echocardiography: comparison with two-dimensional
echocardiography and nuclear magnetic resonance imaging
in normal subjects. J Am Coll Cardiol. 1993;22(1):25870.
72. King DL, Gopal AS, Schroeder KM, et al. Ratio of infarct
subtended volume to surface area by 3D echocardiography:
In vivo measurement of infarct expansion and aneurysm
formation. In: Proceedings of the Computers in Cardiology
1993; September 58, 1993, London, UK. IEEE Computer
Society Press. Los Alamitos, CA. pp. 1720.
73. Maffessanti F, Lang RM, Corsi C, et al. Feasibility of left
ventricular shape analysis from transthoracic real-time
3-D echocardiographic images. Ultrasound Med Biol. 2009;
35(12):195362.
1173
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1175
CHAPTER 55
Newer Aspects of Structure/Function
to Assess Cardiac Motion
Gerald Buckberg, Navin C Nanda, Julien IE Hoffman, Cecil Coghlan
Snapshot
State-of-the-Art
INTRODUCTION
Cardiac motion, until recently, had been thought to follow
the observations of William Harvey, who dissected cadaver
hearts and deduced that the heart underwent constriction
for ejection and dilation for filling, acting like a water
bellows. Keith1 delivered the classic article on structure
and function during presentation of his 1918 Harveian
Lecture, and called Harvey the functional anatomist
who emphasized that we cannot claim to have mastered
the mechanism of the human heart until we have a fundamental explanation of its architecture. Keith described the
cardiac architecture to contain circumferential and helical
fibers, as he perhaps relied upon the observations of Lower2
in the 1600s describing that the cardiac apex showed helical
fibers, or Senac in the 1700s,3 who defined an internal
helix and surrounding transverse circumferential fibers, or
Krehls 1800s description4 of its powerful circumferential
fibers that cause cardiac constriction during ejection.
Physiological recordings of pressure and flow have clearly defined the impact of ventricular performance on these
variables, but their cause is determined by the function
of the underlying ventricular structure (Figs. 55.1A to C).
The Septum
Other Consideraons
1177
B1
1178
B2
B3
B4
Figs 55.1A to C: (A) Currently accepted time frames of systole and diastole, with measurements of intravascular pressure in the aorta,
left ventricle (LV), left atrium (LA), and LV volume, together with their impact on the mitral and aortic valves. Aortic flow occurs between
the two intervals that define ejection. The physiological phases of cardiac cycle that include isovolumic contraction, ejection, isovolumic
relaxation (to be questioned in this report), rapid and slow filling, and atrial contraction are shown; (B) Two-dimensional images of the
LV in a longitudinal view that shows the normal sequence of narrowing, shortening, lengthening, and widening of the ventricular cavity
during a normal cardiac cycle. Images were obtained by epicardial imaging in an open-chest porcine preparation. The phases of the cardiac cycle include end-diastolic state (B4), isometric phase (B1), ejection (B2), and isovolumic phase (B3). The broken-line markers are
within the ventricular cavity and define the transverse (between the midendocardial walls) and the longitudinal (from apical endocardium
to a line across the mitral annulus) dimensions. Muscle thickness is shown by the dark area adjacent to these intracavity dimensional
lines. The pale color is the cavity. The predominant changes exist with muscular thickening that narrows and widens the cavity rather
than the external wall dimensional changes. Note progressive muscular thickening (evaluated by wider distance between epicardial and
endocardial lines as myocardial mass narrows and shortens for ejection), together with maintained thickness as heart lengthens during
the rapid filling phase before substantial widening; (C) Twist of the heart: clockwise (below baseline) and counterclockwise (above baseline) motions of the base and apex, respectively, during the cardiac ejection and filling periods are represented in rotational degrees with
the use of speckle tracking with marker placed at the LV endocardial surface (Echopac PC V 6, GE Healthcare, Milwaukee, WI, USA).
The relationships between the initial uniform and then reciprocal twisting motions of the base and apex during the pre-ejection, ejection,
and rapid and slow filling periods are explained in the text.
1179
Figs 55.2A and B: (A) Magnetic resonance imaging (MRI) phase contrast velocity mapping (tissue phase mapping) of systolic and diastolic
cardiac frames with a temporal resolution of 13.8 ms during free breathing in a healthy volunteer. All motions are described in the text; the
arrows show the clockwise (marker to right) and counterclockwise
(marker to left) directions of transmural twisting motion during the
short-axis view and are obtained during isovolumic contraction, midsystole, isovolumic relaxation phase, and slower filling in mid-diastole;
(B) Differences in mean values for tracing radial, tangential, and longitudinal velocity motion, each 13.8 ms, for 12 volunteer subjects in whom
basal, mid, and apical segments are analyzed. Values above zero line
indicate contraction, clockwise motion, and shortening; below the zero
line, values define expansion, counterclockwise motion, and lengthening. The line expansion time is end systole (ES), with an average 320 ms
time frame. Note early radial expansion in basal segment (a), reversal of
twisting before end of systole (b), and supplemental late counterclockwise base motion during systole (c).
1180
Figs 55.3A and B: (A) Myocardial fiber organization. (a) Mall and MacCallums suggestion of bundles, which include deep (circular)
and oblique bulbospiral tracts. (b) Rushmers functional model, which includes the central transverse constrictor muscle and oblique
clockwise and counterclockwise layers. (c) Torrent-Guasps fiber trajectory model showing an upper transverse circumferential muscle
(or basal loop) surrounding the oblique right- and left-handed helical apical loop; (B) (a) Diffusion tensor magnetic resonance imaging
(DTMRI) studies where water is diffused parallel to fiber orientation, showing a helical positive or right-handed helix or clockwise (red)
and negative or left-handed helix or counterclockwise (yellow) muscle of myofibers reflecting circumferential or horizontal with a zero
helix angle. Note absence of circumferential or circular fibers in the septum, and how these zero angle helix fibers encircle the left and
right ventricles. (b) Dissected heart showing the circumferential or basal loop fibers encircling the left and right ventricles that are not
present in septum, and overlapping left and right helical fibers of the apical loop in septum.
STATE-OF-THE-ART
The underlying myocardial muscle mass is composed
of helical and circumferential fibers, even though their
origins are uncertain.6,7,19 There is general agreement
from DTMRI studies that the basal two thirds of the left
ventricular (LV) free wall contains three layers of muscle.
These correspond to the layers defined by Streeter20 who
found that the inner 20% of the wall had subendocardial
fibers with an average angle of about +60, where the
positive sign indicates counterclockwise rotation above
the equator, the outer 25% of the wall had subepicardial
1181
COMPOSITE OF STATE-OF-THE-ART
REPORTS
Prior imaging reports only address the helical component as a smooth change from a left-handed helix in
the subepicardium into a right-handed helix in the
subendocardium, without considering actions of the
anatomical circular fiber structure that remains the
centerpiece of anatomical descriptions (Fig. 55.5),4,3739
and whose presence is further confirmed by DTMRI
recordings25,26 (Fig. 55.3B). The mechanisms for twisting,
whereby the apex rotates counterclockwise and the
base rotates clockwise, has been based upon the Taber
model of a single helical layered architecture,40 where
obliquely aligned muscle fibers are embedded in an
isotropic matrix. This engineering design states that
torsion develops within each layer so that epicardial fiber
contraction rotates the apex in a counterclockwise and the
base in clockwise direction, while subendocardial region
contraction will rotate the LV apex and base in exactly the
opposite directions. In contrast, this description of torsion
within each right- and left-handed helix differs from the
current mechanistic descriptions by showing that torsion
develops between each arm of the helix, whereby there
is clockwise motion of the entire right-handed arm and a
counterclockwise motion of the entire left-handed arm16,17
(Fig. 55.6).
When both helical layers contract simultaneously
during ejection, the larger radius of rotation for the
outer epicardial layer provides a mechanical advantage
1182
B1
B2
B3
B4
Figs 55.4A and B: (A) Unscrolled myocardial band model of Torrent-Guasp that contains a circumferential basal loop and a helical apical loop.
Note (1) the transverse basal loop fiber orientation (be) representing circumferential fibers, and (2) the right- and left-handed apical loop helix
with predominantly oblique fibers, and (3) myocardial fold in (e) showing basal midwall twist to form the apical loop. The unfolded basal loop (d)
contains a right segment (RS) and left segment that surround the left and right ventricles. The septum does not have circumferential fibers. The
apical loop has helical fibers that form the right-handed helical arm or descending segment (DS) and left-handed arm or ascending segment
(AS). The unfolded myocardial band in (e) extends between the pulmonary artery (PA) and the aorta (Ao). Note (a) the intact heart contains
a circumferential basal loop wrap that surrounds the apical loop comprising helical fibers; (B) Architectural fiber orientation of (B1) intact heart
in upper left with circumferential fibers surrounding the inner helical fibers, (B2) detached circumferential fibers (basal loop) in upper right with
predominantly horizontal fibers compared with the conical apical loop containing right- and left-handed helical fibers in a helical design, (c) with
these segments super-imposed (top image); when the segments are separated (below), (B3) the right-handed helix or descending segment
(lower left) connected to the myocardial fold with oblique fibers aimed toward apex, and (B4) overlying left-handed helix or ascending segment
(lower right) with longer oblique fibers coursing toward aorta connection. This fiber orientation is used in all subsequent anatomical drawings.
1183
Fig. 55.5: Conceptual cartoons of myofiber structural orientation from imaging and anatomy reports. The imaging drawing (left) separates the
left ventricle into a deep endocardium with right-handed helical clockwise fibers and a superficial epicardium with left-handed helical counterclockwise fibers. There is no circumferential or circular muscle. The anatomical drawing (right) displays similar right- and left-handed helical
arms in the deep endocardium and superficial epicardium regions but adds the prominent component of thick circular or circumferential fibers
that reflect the Triebwerkzeug described by Krehl.4 These circular fibers are considered to be constrictor fibers by anatomists.
DEFINITIONS
Rotation is the circular or angular movement of the
LV about its long axis, and by convention is defined as
clockwise or counterclockwise when looking up at the
heart from the foot of a supine patient. If the whole LV
rotates en masse, there is no torsion. Conversely, if apex
and base rotate in opposite directions, then torsion can
be assessed by the angular difference between them
(Fig. 55.8).
Twisting describes these differences without reference to a long-axis measurement. Untwisting expresses
the return of cardiac shape to its initial resting position.
Torsion defines the difference between the rotation of
1184
Fig. 55.6: Structural reasons for torsion from bioengineering drawings (above) and anatomical structure (below). Comparable findings during torsion development are reported in bioengineering studies under conditions where myocardium structure is displayed
as either cylindrical (upper left) or conical (upper right), as the right-handed helical arm or deeper clockwise endocardium and is
covered by a left-handed helical or counterclockwise arm. Torsion is described as developing within each arm as shown by the
arrows in the cylinder on the right, and each arm develops clockwise and counterclockwise motion as shown on the left. The clockwise
layer is R1, counterclockwise layer is R2, and its larger torque causes apical counterclockwise rotation during torsion. Below, the anatomical structure shows a right-handed helical arm with clockwise motion (lower right) and a left-handed helical arm with counterclockwise motion (lower right) and these arms are called the descending and ascending segments of the apical loop. Torsion is described as
developing between helices, as the entire right- and left-handed helix move in different directions.
the base and apex of the LV relative to the long axis, exists
beyond the interval for systolic ejection, is measured
in degrees, and defining its duration is a vital part of its
measurement.
1185
C
Figs 55.7A to C: Motion of apex and base during isovolumic contraction by speckle tracking imaging (STI; upper) and magnetic resonance imaging (MRI) studies. (A) The STI study shows counterclockwise (above baseline) and clockwise motions of the base and apex,
respectively, during the isovolumic contraction. The speckle tracking with marker is placed at the left ventricular (LV) endocardial surface (Echopac PC V 6, GE Healthcare, Milwaukee, WI, USA). Tracings from Aman Mahajan laboratory; (B) MRI studies showing global
counterclockwise motion of the apex and base during isovolumic contraction in both studies. Tagged MR images were acquired on a
1.5 T whole body MR scanner (Magnetom Sonata, Siemens, Erlangen, Germany) with a temporal resolution of 14 ms. The hatched line
following twisting (apex counterclockwise and base clockwise) marks peak apical rotation that exists just prior to the postejection isovolumic interval. Note: apex begins clockwise motion at this stage and prolongation of clockwise base rotation; global clockwise motion
occurs during this postejection isovolumic interval; (C) Velocity vector imaging (VVI) short-axis views of endocardial rotational motion of
six segments at the apex and base employing the (Sequoia 512, Siemens, Mountain View, CA, USA 4.0 MHz transducer) derived from
three-dimensional (3D) images displays counterclockwise motion of the apex and base during the isovolumic contraction (IVC) phase.
The hatched purple lines show both the end of the IVC phase where AVo is aortic valve opening, and the separated purple hatched lines
between AVc or aortic valve closure and MVo or mitral valve opening show the postejection isovolumic interval.
1186
Fig. 55.8: Ventricular torsion displayed by speckle tracking imaging (STI) on left side (From Aman Mahajan laboratory) and magnetic resonance imaging (MRI) on right side (From Jurgen Hennig
laboratory), where twisting motions between the left ventricular
apex and base are displayed. STI study shows counterclockwise
(above baseline) and clockwise motions of the apex and base,
respectively, during the cardiac ejection. The speckle tracking with
marker is placed at the left ventricular (LV) endocardial surface
(Echopac PC V 6, GE Healthcare, Milwaukee, WI, USA). MRI
phase contrast velocity mapping (tissue phase mapping) of systolic ejection cardiac frames with a temporal resolution of 13.8 ms
during free breathing in a healthy volunteer shows arrows that
demonstrate clockwise (marker to right) and counterclockwise
(marker to left) directions of transmural twisting motion during the
short-axis view.
Torsion
The presystolic isovolumic interval is followed by
ventricular ejection, whereby torsion develops as the
1187
B
Figs 55.9A and B: (A) On the left side is cranial view of the model of the helical ventricular myocardial band showing how the circular
and circumferential fibers or basal loop surrounds and embraces the conical right- and left-handed helix or apical loop. Note that (a)
circumferential fiber muscle thickness is greatest in the left component or segment, and thinner in the right component or segment and
(b) there are no circumferential fibers in the septum. On the right side are VVI images of isovolumic contraction (Sequoia, 512, Siemens,
Mountain View, CA, USA; 4.0 MHz transducer), where there is shortening of the entire circumference of basal loop, and of the righthanded helical armor descending segment. No left-handed arm or ascending segment shortening occurs, yet right-sided upper septum
motion exists in area of uncovered noncontracting left-handed helix or ascending segment as shown in B images; (B) Topographical view
of septum architecture in the wrapped heart where the right ventricle is intact (left side) and unwrapped form where the circumferential
fibers are separated. Note that the apical loop has a septum segment above the overlap of right- and left-handed helical fibers, where
the left-handed arm or ascending segment is the only muscle mass. This segment does not shorten during the isovolumic contraction,
and is the only segment that shortens during the postejection isovolumic phase (Fig. 55.8A, left panel and Fig. 55.11B, in a figure).
1188
1189
1190
Fig. 55.10: Beginning of torsion (upper left) with co-contraction of base and entire helix. There is essentially no longitudinal ventricular
shortening at this time. Note (a) twisting septum with upper septum showing left-sided motion of the right-handed arm of the descending
segment and lower septum, and lower lateral wall has right-sided motion of left-handed arm or ascending segment, and (b) upper lateral
wall has essentially no motion as it is compressed by shortening circular basal muscle, which does not exist in the septum, and (c) the
lower lateral wall has no circumferential compression and shows leftward motion of left-handed arm or ascending segment. Slightly later
in torsion (upper right), with more twisting of septum, and increased left-sided motion of lateral wall that is occupied by the more fully
contracting left-sided arm or ascending segment. The lower images show the responsible architecture, whereby the lower left shows
the wrapped heart, where the uncovered left-handed helix displays more prominent counterclockwise motion. The lower right displays
unwrapped heart to show how the circumferential muscle covers the same upper lateral wall to exert a compressive force and limit early
rightward counterclockwise motion.
1191
C
Figs 55.11A to C: (A) Velocity vector imaging forces during maximal torsion during ejection as longitudinal shortening occurs. Vector
angulation is directed toward the apex, as the helical left- and right-sided arms dominate to change force direction toward the apical vortex, despite ongoing circumferential or circular muscle shortening; (B) Drawing of simulated cardiac anatomy with circumferential wrap
and internal helix (lower left) with spiral motion for ejection (in center) and suction (lower right). The outward spiral forces with an apical
vortex during ejection would expand the basal wall laterally to potentially cause explosion. Lower right shows suction where inward
forces at the base would cause implosion. This circumferential wrap at the base becomes a buttress to prevent these events, and this
configuration resembles a gothic cathedral (upper center) where the buttress protects the downward forces of the dome from loss of the
base due to downward forces from the tip at the peak of the dome; (C) This drawing shows normal anatomy (upper left) with right and
left segments of basal loop [right segment (RS) and left segment (LS)], and right and left helical arms of apical loop or descending and
ascending segments [descending segment (DS) and ascending segment (AS)]. The bottom shows the coils in the right-handed helix
arms in diastole, ejection, and isovolumic phase. Note that (a) both shorten during ejection, but the descending segment is stronger,
(b) ascending segment becomes more horizontal as spiral shortens, and (c) ascending segment continues shortening during isovolumic
phase and causes elevation or lengthening. This action mirrors cobra shown in upper right that lengthens as its spiral becomes elongated in its pose before striking (Fig. 55.11A).
1192
B
Figs 55.12A and B: (A) On the left, tracings of endocardial and epicardial sonomicrometer crystals placed into the fiber orientation
pathways of the right- and left-handed helix, or descending and ascending segments in anterior myocardium of open-chest pig. The
solid line shows the beginning and ending of the right-handed helix shortening, and the hatched lines show the left-handed helix or
ascending segment. The postejection isovolumic phase (within yellow color overlay) shows (a) approximately 80 ms time hiatus, and
(b) lengthening of the right-handed helix as the left-handed helix or ascending (Asc.) anterior fibers are still shortening; the left ventricle
(LV) pressure and dP/dt tracings indicate the timing. On the right is unfolded myocardial architecture showing left- and right-handed
or ascending and descending segments of the helical ventricle, surrounded by the circumferential muscle of the basal loop. Note that
(1) central ventricular cavity is composed of overlapping left- and right-handed helices or ascending and descending segment fibers in
the septum region, (2) left-handed helix wraps around and overlaps the right-handed helix in septum, and (3) absence of overlap in the
lateral wall, which is composed of the left-handed helix or ascending segment. The lack of overlap in the left-handed helix or ascending
segment also occurs in the septum, below the aortic valve, as displayed in Fig. 55.9B; (B) In (a), beginning of postejection isovolumic
phase with right-sided motion of the upper septum, where there is ongoing shortening of the left-handed arm or ascending segment
without overlap of right-sided arm or descending segment. Simultaneously, there is essentially no motion of the lower septum or lateral
wall, where the circular and right-handed arm and descending segment fibers just stopped shortening. In (b), slightly later in postejection
isovolumic phase, there is left-sided motion of the septum and lateral wall due to recoil of the circumferential or circular basal muscle,
and the upper septum now shows similar left-sided movement despite the ongoing shortening of the left-sided arm or ascending segment that just showed right-sided motion before these recoiling forces became dominant. In (c), end of postejection isovolumic phase
with both septum and lateral wall showing left-sided movement as dominant recoil exists in the circumferential or circular base, which
thereby counteracts the simultaneous counterclockwise recoil of both the right-handed helical arm or descending segment and ongoing
shortening with counterclockwise motion of the left-handed helical arm or ascending segment.
1193
Figs 55.13A and B: (A) First phase of rapid filling with elongation
(note apical lengthening) and further leftward motion of the septum and lateral wall as there is recoil of the left-handed helical arm
or ascending segment, together with termination of recoil or the
circumferential or circular base and right-handed arm or descending segment whose forces interacted during the preceding postejection isovolumic phase; (B) Completion of the rapid filling phase,
whereby increasing ventricular volume is the dominant force as
the left ventricle (LV) chamber further lengthens and widens. Velocity vector imaging (VVI) displays expansion and outward velocities because filling forces overcome recoil action in septum and
lateral wall.
Rapid Filling
Untwisting has two components, as the initial unwinding
occurs during the postejection isovolumic interval and
is dissociated from the untwisting, causing rapid filling
but the causes are tightly interrelated.66 Early untwisting
during the postejection isovolumic interval is the reason
for subsequent suction, despite this temporal separation;
recoil continues from a different cause (the ascending
segment) during the subsequent phase of rapid filling
that develops after ventricular pressure falls below atrial
pressure (Figs 55.13A and B). The first phase of untwisting
is characterized by transmural clockwise motion that is
initially caused by dominant recoil of circular muscle
during this postejection isovolumic interval. The second
phase develops from elastic recoil of compressed titin coils
within the left-handed helix or ascending segment fibers.
The interaction of these dual recoiling forces is critical for
suction, because 5060% of untwisting normally occurs
before the rapid filling phase.64,67 Moreover, a fundamental
component relates to the normal 80 ms timing hiatus
between the completion of shortening of the descending
segment and the later completion of shortening in the
ascending segment. Suction increases if inotropic drugs
1194
THE SEPTUM
The ventricular septum is a thick structure composed
of discrete muscular bands that separate the LV and RV.
The septum comprises approximately 40% of ventricular
muscle mass and contributes to biventricular cardiac
function.68 Analysis of this structure/function relationship
requires a full understanding of how existing normal
anatomical form translates into hemodynamic performance. Satisfactory accomplishment of this task shall
answer the 1790 supposition of Weber,19 who indicated
that actions of muscular heart would not be understood
until the muscle bundles of the septum are clarified.
Our initial experimental evaluation of septal structure/
relationships was acquired by use of sonomicrometer
crystal measurements that demonstrated how fiber
orientation determines the maximum rate of systolic
shortening. Findings validated the hypothesis that the
configuration of septum anatomy conformed to the
descending and ascending segments of the HVMB, as
described by Torrent-Guasp11 (Figs 55.3 and 55.4). This
spatial composition has been recently supported by
DTMRI recordings (Figs 55.14A to C).25,26 Oblique fibers
of the endocardial regions of the left and right sides of the
septum displayed the same functional characteristics that
exist within in the free LV wall, thereby confirming the
spatial structural configuration required for development
of twisting.
1195
Figs 55.15A and B: (A) Cross-section images demonstrating the oblique crisscross endocardial and epicardial fibers contained within
a circumferential midseptal wall; (B) Computed tomography scans demonstrating the interweaving collagen support of the connective
tissue skeleton that is likely the scaffold for reciprocally oblique septal muscular fibers. Note the space between the two septum regions.
1196
B1
B2
B3
B4
1197
Fig. 55.17: Comparison of ultrasonic crystal tracings of descending and ascending segments of left ventricular (LV) free wall, and
M-mode and Doppler M-mode imaging of the septum. The beginning and end of descending segment shortening and motion (solid lines), and the ascending segment (hatched lines). Strain in the right (red) and left (blue) sides of the septum is noted in systole. M-mode shows displacement of the left and right sides of the septum toward their respective ventricular chambers. Note the
delay of initiation of ascending segment and right septal motion and lengthening of descending segment during phase after ejection
and continuing displacement of the right side of the septum toward the right ventricular (RV) cavity, despite the beginning of LV cavity
expansion. (LV: Left ventricle).
1198
OTHER CONSIDERATIONS
Subendocardial Muscle; Correct
Anatomical Location But Architectural
and Functional Confusion
Subendocardial muscle mass surrounds the LV inner
surface, and drawings14,15,40,51 (Fig. 55.19) left side of the
transmural ventricle imply a circumferential line that
bisects the LV wall to separate the deeper oblique clockwise
fibers of the descending segment or right-handed helix
from the overlying counterclockwise ascending segment
or left-handed helix that occupies the outer LV shell. In
contrast, anatomical studies show that different parts of
right- and left-handed helices form the circumferential
subendocardial muscle.10,11,31,32 This difference conveys
a different functional effect, as previously described
during cardiac motions of the normal heart. The dissected
or unraveled cardiac architecture shown in Figs 55.4
and 55.9 shows that the upper septal oblique orientation
differs from reciprocally oblique fibers of the lower
septum, and that the oblique lateral ventricular wall
subendocardial fiber arrangement mirrors that of the
subepicardium, because there is no overlap of descending
and ascending segment helical fibers (Figs 55.9 and 55.10).
A1
A2
A3
A4
1199
1200
Fig. 55.19: The left drawing shows a bioengineering concept of circumferentially overlapping endocardium and epicardium, whereby the
endocardium reflects the right-handed helical arm and surrounds the left ventricular (LV) inner surface. The right architectural reflection
of the anatomical endocardium shows that it is formed by both the right- and left-handed helical arms, and has fiber pathways that have
both clockwise and counterclockwise directions. This anatomy is described in Figure 55.10.
changing the natural 60 angulations of the right- and lefthanded helical components toward a more horizontal82,83
configuration will geometrically alter functional twisting
and untwisting. For example, Borg recently examined
changes after increased preload and reduced afterload
in patients with mitral insufficiency (whose regurgitation
causes these intrinsic spherical ventricular shape changes)
and demonstrated decreased torsion and reduced
untwisting.84 Moreover, initiation of the untwisting
movement began 23 ms before aortic valve closure. This
reliable echocardiographic finding, thereby poses a series
of questions about responsible muscular mechanisms that
produce such reproducible changes. Untwisting normally
begins during the postejection isovolumic phase, yet mitral
valve insufficiency continues after aortic valve closure58 to
thereby support the functional role of ongoing ascending
segment contraction during an interval that previously
was called IVR.
1201
Figs 55.20A and B: Comparison of fiber orientation in the normal heart (above) and the dilated or spherical form (below) where there
is detachment of the circumferential or basal loop with horizontal fibers, and exposure of the normal and spherical configuration of the
right- and left-sided arms of the helical structure. Note that the normal 60 fiber orientation becomes more horizontal in the spherical
configuration and this angulation begins to resemble the more transverse fiber pathways of the circumferential or basal loop.
Clinical Implications
Recognizing how the interdependence of torsion
and untwisting relates to underlying mechanics and
Diastolic Dysfunction
The term diastolic dysfunction has been used because
heart failure occurs in patients with normal ejection
fraction, implying that the problem is diastolic in origin.
However, Tan86 has done an extensive echocardiographic
analysis and emphasized that it is not an isolated diastolic
disorder; each patient displays combined systolic and
1202
Myocyte Factors
Calcium-Related Factors
diastolic abnormalities, particularly involving ventricular
twist and deformation (strain) patterns leading to reduced
ventricular suction, delayed untwisting, and impaired
early diastolic filling.87,88 These observations emphasize
the interdependence of twisting and untwisting. Diastolic
dysfunction has clear echocardiography characteristics
relating to changes in the velocity waves during rapid
filling and atrial contraction. The role of the reported
increased untwisting during early diastolic dysfunction
is uncertain because the E-wave is reduced and impaired
filling occurs.16,89 Diastolic dysfunctions characteristic
impaired untwisting is associated with either (a) increased
torsion and preserved ejection fraction or (b) reduced
torsion from reduced systolic function.
The underlying problem is prolonged shortening of
the right-handed helical arm or descending segment
that causes extended torsion duration with resultant
compromise of the vital postejection isovolumic
phase timing hiatus. The keynote echocardiographic
observation is loss of longitudinal strain, a process caused
by the prolonged descending segment contraction causing
prolonged torsion, so that there is a delay in allowing the
noncontracting descending segment to become a fulcrum
for lengthening. Several reasons exist for this descending
segment prolongation, and the resultant treatment
options are determined by whether the causative factor
relates to (a) regional muscle anatomy, (b) physiological
calcium flux, or (c) geometric interruption of normal fiber
orientation by cardiac dilation.
Dilated Cardiomyopathy
Geometric reasons for diminished torsion and impaired
untwisting become apparent as the normal conical
ventricular shape becomes spherical in dilated cardiomyopathy, as increased sphericity index is a primary
determinant of abnormal twisting and associated diastolic
dysfunction.79 Geometric changes thereby become the
1203
Figs 55.22A to D: Sonomicrometer crystal tracings of hiatus between termination of right- and left-handed helix or descending and
ascending segment contraction during the isovolumic phase in the normal heart. A yellow shade defines this interval, and there is
recording of left ventricular pressure and dP/dt. (A) Normal or control intervals; (B) Bulging of both segments during ischemia or temporary coronary occlusion, without shortening; (C) 15 minutes after reperfusion shows reduced shortening, prolongation of right-handed
helix, or descending segment contraction that markedly reduces the hiatus between termination of the right- and left-handed helices
or descending and ascending shortening; (D) Diastolic dysfunction is percentage prolongation of hiatus between end of shortening
of right-handed helix (descending segment) and left-handed helix or ascending segment. Prolonged right-handed helix or descending
shortening defines this interval. The treated animals received cariporide, a sodium hydrogen ion inhibitor called HOE pretreatment.
Control values are shown below. Values expressed as mean SEM. Note return of normal hiatus following this intervention (*P < 0.05
HOE pretreatment vs no treatment).
Pacing Factors
Cardiac motion studies show that normal cardiac
movement is sequential due to spread of the electrical
impulse from its earliest action within the conduction
1204
CONCLUSION
Ventricular torsion is due to the twisting of the ventricle
during systole, and its subsequent untwisting is the prelude
1205
B
to subsequent diastolic filling. These interdependent
rotational events arise from the mechanical actions and
timing relationships of the hearts underlying circular and
helical muscle pathways. Explanation of the presystolic
IVC period is essential for analysis of these interactions.
Circular fibers dominate to cause pre- and post-twisting
net or global counterclockwise and clockwise movement,
whereas the helical fibers govern torsion. Normal
1206
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in stunned myocardium: a state of abnormal excitationcontraction coupling that is limited by Na+-H+ exchange
inhibition. Eur J Cardiothorac Surg. 2006;29(Suppl 1):
S10714.
64. Dong SJ, Hees PS, Siu CO, et al. MRI assessment of LV
relaxation by untwisting rate: a new isovolumic phase
measure of tau. Am J Physiol Heart Circ Physiol. 2001;
281(5):H20029.
65. Notomi Y, Srinath G, Shiota T, et al. Maturational and
adaptive modulation of left ventricular torsional biomechanics: Doppler tissue imaging observation from infancy
to adulthood. Circulation. 2006;113(21):253441.
1208
84.
85.
86.
87.
88.
89.
90.
91.
92.
93.
94.
95.
96.
97.
98.
99.
100.
101.
102.
103.
1209
CHAPTER 56
Echocardiography in Assessment of
Complications Related to Permanent
Pacemakers and Intracardiac
Defibrillators
Ahmed Almomani, Khadija Siddiqui, Masood Ahmad
Snapshot
Pacemakers/Implantable Cardioverter-Defibrillators
Tricuspid Regurgitation
INTRODUCTION
Over the past decades, technical advances in permanent
pacemakers (PPMs) and implantable cardioverter-defibri
llators (ICDs) have led to a tremendous increase in the
use of these medically important devices. This trend is
likely to continue due to the increased life expectancy of
the population and the increasing number of indications
for their use including placement of PPMs for cardiac
resynchronization therapy (CRT) and ICDs for primary and
secondary prevention of complications from arrhythmias
in patients with left ventricular dysfunction.13 The 11th
World Survey of Cardiac Pacing and ICDs reported that
737,840 new devices were implanted in 2009 worldwide,
with the largest number of new implants, 225,567, in the
United States. These numbers showed a huge increase
compared to a similar survey done in 2005, and the
numbers are expected to be much higher in 2013.4
Myocardial Perforation
NORMAL ECHOCARDIOGRAPHIC
FINDINGS IN PERMANENT
PACEMAKERS/IMPLANTABLE
CARDIOVERTER-DEFIBRILLATORS
Transthoracic echocardiography can be used to visualize
the intracardiac portion of the PPM or ICD lead. Device
Chapter 56: Echo in Assessment of Complications Related to Permanent Pacemakers and Intracardiac Defibrillators
1211
Figs 56.1A to E: Two-dimensional transthoracic echocardiography (2D TTE) with pacer lead shown in modified parasternal
long-axis (A), short-axis (B), apical four-chamber (C), focused
right-sided chambers (D), and subcostal (E) views. Arrowheads
point to the lead, arrow in Figure A points to the tricuspid valve.
(AO: Aortic valve; LA: Left atrium; LV: Left ventricle; RA: Right
atrium; RV: Right ventricle).
1212
Figs 56.2A to D: Real time transthoracic three-dimensional echocardiography (RT3DE) in apical four-chamber view demonstrating the
pacer route and its relation to intracardiac structures in simultaneously obtained multiple views derived from the same data set. Arrowheads point to the lead. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: right ventricle).
TRICUSPID REGURGITATION
The development of significant TR and its progression
over time is an important device-related complication.
Prospective data on TR secondary to PPM and ICD
leads are lacking. Multiple small retrospective studies
and small case series have reported this complication
(Table 56.1). TR in patients with PPMs or ICDs may not
be exclusively caused by the endocardial lead, as preexisting abnormalities such as tricuspid valve (TV) annular
dilatation or pulmonary hypertension may be present.5
A number of different mechanisms of RV intracardiac
lead-related TR have been described. In one retrospective
study, it was noted that in 41 patients with PPM or ICD
lead-induced severe TR requiring surgery, 7 patients had
perforation of the TV leaflet by the PPM or ICD lead, 4 had
lead entanglement in the TV, 16 had lead impingement
of the TV leaflets, and 14 had lead adherence to the TV.6
Chapter 56: Echo in Assessment of Complications Related to Permanent Pacemakers and Intracardiac Defibrillators
1213
Study
Sakai et al. (1987)58
Paniagua et al. (1998)
59
Sample Size
Study Design
Follow-up
18
Prospective
NA
NA
374 cases
Case control
NA
NA
27
Moderate to severe TR
NA
NA
12
(P < 0.0001)
10
Moderate to severe TR
(P = Not significant)
NA
NA
12
Moderate to severe TR
(P = Not significant)
683 controls
Leibowitz et al. (2000)
35
Prospective
41
Retrospective
61
Prospective
87
Retrospective 36 months
NA
32
Moderate to severe
TR3D echocardiography
was used
248
Retrospective 93 days
(range,
23199)
69
NA
410
Retrospective 75 days
(range,
14,367 d)
NA
NA
23
Prospective
48.6 32.7
months
115
Prospective
4.1 0.8
years
10
36
Moderate to severe TR
(P < 0.001)
60
6 3 months
In the same study, severe lead-induced TR causing rightheart failure that required TV surgery accounted for 2.8%
of all TV operations (41 of 1,465 consecutive patients)
between 1993 and 2003 at the Mayo Clinic.
The time course for TR development and progression
after endocardial lead placement in the RV is not well
defined. A large retrospective study reported an increase
in TR acutely after RV lead implantation, and progressive
worsening with time, while other studies have mainly
focused on the chronic effects of the leads on the
valve.7 Pathological studies have demonstrated major
inflammatory changes occurring within the heart only days
after lead implantation. The progression of inflammation
1214
Infection
Initial cases of pacemaker endocarditis were described
in the early 1970s.10 Reported device-related infective
endocarditis (IE) has ranged from 10% to 23% in the
literature.11,12 The incidence of infection following implan
tation of PPMs has been variably assessed, ranging from
0.13% to 19.9%,13,14 with the majority of infections repo
rted to be in the pacemaker generator pocket. Incidence
of infection in ICDs has been reported in the literature
ranging from 0.7% to 1.2%.15,16 Technical advances have
allowed transvenous implantation of ICDs, thereby
eliminating the need for thoracotomy and epicardial lead
placement. These advances have contributed to the overall
decline in infection associated with ICD.
In patients with device-related endocarditis, the
presence of vegetation is limited not only to the TV but
can be found anywhere along the course of the lead,
including the endocardium of the right atrium or RV.17
Echocardiography plays an important role by allowing
direct visualization and measurement of the mass along
with the ability to assess for cardiac involvement. On
echocardiography, vegetations have been defined as
oscillating intracardiac masses on the device leads, valve
leaflets, or endocardial surface, confirmed by imaging in
more than one echocardiographic plane. In these cases,
the valve or lead infection was confirmed by positive
blood or lead tip culture.12,18 Valvular vegetations have
been further characterized on the basis of four physical
properties, as assessed by echocardiography: vegetation
size, mobility, extent, and consistency.18
Chapter 56: Echo in Assessment of Complications Related to Permanent Pacemakers and Intracardiac Defibrillators
1215
Figs 56.4A and B: Real time transthoracic three-dimensional echocardiography (RT3DE) with pacer and en-face view of the tricuspid
valve from the right atrium (A). Pacer and the tricuspid valve from the right ventricle (B), the lead position (arrowhead) in relationship
to the tricuspid valve (arrow).
Thrombus
Venous thrombosis and stenosis have been described
as the most common complications associated with
transvenous pacemaker implantation with incidence
ranging between 30% and 45%.22 Right atrial thrombus
is a rarely reported event, and can present either as an
incidental finding on echocardiogram,23 or as symptoms
of right-sided heart failure,24 obstruction, or embolization
of the pulmonary artery.25 One study described only two
MYOCARDIAL PERFORATION
Myocardial perforation is a relatively rare complication
of endocardial leads of cardiac implantable devices. The
incidence of this complication is estimated to be < 1%.
1216
Fig. 56.5: Two-dimensional transesophageal echocardiography (2D TEE) showing the pacer lead (arrowhead) and attached
mobile thrombus (arrow).
Fig. 56.7: Echocardiography (four-chamber apical view): implantable cardioverter-defibrillator (ICD) lead perforation across the
right ventricular apex. (LV: Left ventricle; PE: Pericardial effusion;
RV: Right ventricle).
Source: Reproduced with permission from Sassone B, Gabrieli L,
Boggian G, Pilato E. Management of traumatic implantable cardioverter defibrillator lead perforation of the right ventricle after car
accident: a case report. Europace. 2009;11(7):9612.
Fig. 56.6: Real time transthoracic three-dimensional echocardiography (RT3DE) of the pacer lead shown in Figure 56.5; right
ventricle (RV) lead (arrowhead) with a thrombus (arrow).
Chapter 56: Echo in Assessment of Complications Related to Permanent Pacemakers and Intracardiac Defibrillators
1217
Characteristics
Total Number = 35
Mean Age
Gender:
Male
16 (45.7%)
Female
19 (54.3%)
Type of device:
Pacemaker
19 (54.3%)
ICD
15 (42.8%)
NA
1 (2.9%)
Active
24 (68.6%)
Passive
6 (17.1%)
NA
5 (14.3%)
Yes
24 (68.6%)
No
4 (11.4%)
NA
7 (20.0%)
Fig. 56.8: Three-dimensional echocardiogram of an apical fourchamber view, demonstrating the pacemaker lead tip (arrow)
going through the interventricular septum (arrowhead); (LV: Left
ventricle; RV: Right ventricle).
Source: Reproduced with permission from Daher IN, Saeed M,
Schwarz ER, Agoston I, Rahman MA, Ahmad M. Live threedimensional echocardiography in diagnosis of interventricular septal perforation by pacemaker lead. Echocardiography.
2006;23(5):4289.
1218
Figs 56.9A and B: Three dimensional (3D) segmental timevelocity curves of left ventricle (LV) before pacing, left ventricular ejection fraction (LVEF) 48.5%, SDI 2.7% (A) and after right ventricle (RV) pacing, ejection fraction (EF) 45.7%, SDI 10.9% (B), showing
increased LV dyssynchrony and decrease in LVEF after pacing.
CONCLUSION
The increasing indications and uses for implantable
cardiac devices have led to a continuous increase in the
number of implanted devices each year. Implantation
of endocardial leads for these devices can cause many
delayed complications. Some of the complications are
mechanical and related to the presence of foreign body
and its interaction with the valves and endomyocardium,
for example, perforation, infection, and thrombosis, while
others are related to the electrical pacing of the myocardium
and conduction abnormalities, for example, dyssynchrony
and TR. It is important to have a high index of suspicion
to diagnose these complications, using the appropriate
imaging modality. Based on the preceding review, it is
clear that echocardiography plays an important role in the
diagnosis of the device-related complications. Both 2D TTE
and TEE provide extremely useful diagnostic information
that is helpful in detecting the endocardial lead-related
complications. Real time 3D echocardiography is a novel
technique that can precisely track the intracardiac route of
the lead and accurately detect most of the pacemaker/ICD
lead-related complications.
Chapter 56: Echo in Assessment of Complications Related to Permanent Pacemakers and Intracardiac Defibrillators
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1. Uslan DZ, Tleyjeh IM, Baddour LM, et al. Temporal trends
in permanent pacemaker implantation: a populationbased study. Am Heart J. 2008;155(5):896903.
2. Hammill SC, Kremers MS, Kadish AH, et al. Review of the
ICD Registrys third year, expansion to include lead data
and pediatric ICD procedures, and role for measuring
performance. Heart Rhythm. 2009;6(9):1397401.
3. Svendsen A. The current status of cardiovascular disease in
Canadaa call to action. Can J Cardiovasc Nurs. 2004;14(1):
57.
4. Mond HG, Proclemer A. The 11th world survey of cardiac
pacing and implantable cardioverter-defibrillators:
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project. Pacing Clin Electrophysiol. 2011;34(8):101327.
5. Al-Mohaissen MA, Chan KL. Prevalence and mechanism
of tricuspid regurgitation following implantation of endo
cardial leads for pacemaker or cardioverter-defibrillator. J
Am Soc Echocardiogr. 2012;25(3):24552.
6. Lin G, Nishimura RA, Connolly HM, et al. Severe sympto
matic tricuspid valve regurgitation due to permanent
pacemaker or implantable cardioverter-defibrillator leads.
J Am Coll Cardiol. 2005;45(10):16725.
7. Seo Y, Ishizu T, Nakajima H, et al. Clinical utility of
3-dimensional echocar
diography in the evaluation of
tricuspid regurgitation caused by pacemaker leads. Circ J.
2008;72(9):146570.
8. Becker AE, Becker MJ, Claudon DG, et al. Surface
thrombosis and fibrous encapsulation of intravenous pace
maker catheter electrode. Circulation. 1972;46(2):40912.
9. Schnabel R, Khaw AV, von Bardeleben RS, et al. Assessment
of the tricuspid valve morphology by transthoracic real
time 3D-echocardiography. Echocardiography. 2005;22(1):
15-23.
10. Schwartz IS, Pervez N. Bacterial endocarditis associated
with a permanent transvenous cardiac pacemaker. JAMA.
1971;218(5):7367.
11. Arber N, Pras E, Copperman Y, et al. Pacemaker endocarditis.
Report of 44 cases and review of the literature. Medicine
(Baltimore). 1994;73(6):299305.
12. Sohail MR, Uslan DZ, Khan AH, et al. Infective endocarditis
complicating permanent pacemaker and implantable
cardioverter-defibrillator infection. Mayo Clin Proc.
2008;83(1):4653.
13. Conklin EF, Giannelli S Jr, Nealon TF Jr. Four hundred
consecutive patients with permanent transvenous
pacemakers. J Thorac Cardiovasc Surg. 1975;69(1):17.
14. Bluhm G. Pacemaker infections. A clinical study with
special reference to prophylactic use of some isoxazolyl
penicillins. Acta Med Scand Suppl. 1985;699:162.
15. Moss AJ, Zareba W, Hall WJ, et al.; Multicenter Automatic
Defibrillator Implantation Trial II Investigators. Proph
ylactic implantation of a defibrillator in patients with
myocardial infarction and reduced ejection fraction. N
Engl J Med. 2002;346(12):87783.
1219
1220
49. Manolis AS. The deleterious consequences of right
ventricular apical pacing: time to seek alternate site pacing.
Pacing Clin Electrophysiol. 2006;29(3):298315.
50. Karpawich PP, Rabah R, Haas JE. Altered cardiac histology
following apical right ventricular pacing in patients with
congenital atrioventricular block. Pacing Clin Electro
physiol. 1999;22(9):13727.
51. Thambo JB, Bordachar P, Garrigue S, et al. Detrimental
ventricular remodeling in patients with congenital
complete heart block and chronic right ventricular apical
pacing. Circulation. 2004;110(25):376672.
52. Tse HF, Yu C, Wong KK, et al. Functional abnormalities
in patients with permanent right ventricular pacing: the
effect of sites of electrical stimulation. J Am Coll Cardiol.
2002;40(8):14518.
53. Wang H, Shuraih M, Ahmad M. Real time three-dimen
sional echocardiography in assessment of left ventricular
dyssynchrony and cardiac resynchronization therapy.
Echocardiography. 2012;29(2):1929.
54. Sgaard P, Egeblad H, Kim WY, et al. Tissue Doppler
imaging predicts improved systolic performance and
reversed left ventricular remodeling during long-term
cardiac resynchronization therapy. J Am Coll Cardiol.
2002;40(4):72330.
55. Galderisi M, Cattaneo F, Mondillo S. Doppler echocar
diography and myocardial dyssynchrony: a practical
update of old and new ultrasound technologies. Cardiovasc
Ultrasound. 2007;5:28.
56. Marsan NA, Bleeker GB, Ypenburg C, et al. Real-time threedimensional echocardiography permits quantification of
left ventricular mechanical dyssynchrony and predicts
acute response to cardiac resynchronization therapy. J
Cardiovasc Electrophysiol. 2008;19(4):3929.
57. Kapetanakis S, Kearney MT, Siva A, et al. Real-time threedimensional echocar
diography: a novel technique to
quantify global left ventricular mechanical dyssynchrony.
Circulation. 2005; 112(7):9921000.
58. Sakai M, Ohkawa S, Ueda K, et al. [Tricuspid regurgitation
induced by transvenous right ventricular pacing: echocar
diographic and pathological observations]. J Cardiol.
1987;17(2):31120.
59. Paniagua D, Aldrich HR, Lieberman EH, et al. Increased
prevalence of significant tricuspid regurgitation in patients
with transvenous pacemakers leads. Am J Cardiol. 1998;
82(9):11302, A9.
60. Leibowitz DW, Rosenheck S, Pollak A, et al. Transvenous
pacemaker leads do not worsen tricuspid regurgitation:
a prospective echocardiographic study. Cardiology. 2000;
93(1-2):747.
61. Kucukarslan N, Kirilmaz A, Ulusoy E, et al. Tricuspid
insufficiency does not increase early after permanent
implantation of pacemaker leads. J Card Surg. 2006;21(4):
3914.
62. Kim JB, Spevack DM, Tunick PA, et al. The effect of
transvenous pacemaker and implantable cardioverter
defibrillator lead placement on tricuspid valve function:
an observational study. J Am Soc Echocardiogr. 2008;21(3):
2847.
Chapter 56: Echo in Assessment of Complications Related to Permanent Pacemakers and Intracardiac Defibrillators
1221
CHAPTER 57
Echocardiographic Evaluation of
Ventricular Assist Devices
Peter S Rahko
Snapshot
Reverse Remodeling
Types of Devices
INTRODUCTION
Mechanical circulatory support began in the 1960s when
Michael DeBakey and his colleagues first began conceiving of a left ventricular assist device (LVAD). Early devices
were pneumatic with an external drive system, and
the first device was implanted in 1963 in a patient who
suffered a cardiac arrest. By 1966, a second pneumatic,
pulsatile LVAD was developed and utilized in a patient.1,2
Considerable development in pulsatile LVADs, namely,
devices that had a chamber that filled and then ejected
using a pusher plate design, expanded throughout
the 1970s and into the 1980s. The first Food and Drug
Administration (FDA)-approved intracorporeal LVAD was
the Thoratec HeartMate XVE. Initially, it was pneumatically
powered but later became electrically powered. This was
the first realistic device that gave patients independence
since everything was internal except a driveline that came
out from a skin tunnel, and attached to a controller and
battery pack. For the first time, patients could be relatively
mobile and realistically go home and even go back to work
in some situations. The superiority of device therapy over
medical therapy was established in the REMATCH trial for
end-stage heart failure patients.3 However, the pulsatile
chamberpusher plate design had many moving parts,
Explantaon
1223
Figs 57.1A to D: (A) The Thoratec HeartMate II draws blood from the left ventricle (LV) apex through the inflow cannula into the pump
and then ejects blood through the outflow cannula to the central aorta; (B) Closer view showing the impellar design. (Used with permission of Thoratec); (C) Chest X-ray of patient with a HeartMate II showing inflow cannula position and relative position of the left ventricular assist device (LVAD) in the chest; (D) Three-dimensional reconstruction of a chest CT scan of same patient as (C). Note position of
cannula and in this case the tortuous course of the outflow cannula.
A
Figs 57.2A and B
1224
Figs 57.2A to D: Example of a centrifugal pump, the HeartWare device. (A) Relative size is small; (B) The device open shows the
rotating disk that spins blood outward from the center; (C) Diagram showing relative location of device at the left ventricle (LV) apex and
the outflow cannula to the central aorta; (D) View showing relative location in the chest of the components of the device.
Source: Used with permission from HeartWare International.
conceived as bridge to recovery devices to allow temporary support of patients expected to recover from a
temporary but severe clinical situation. Devices designed
for this purpose are in clinical use today and typically are
percutaneously deployed rapidly in urgent situations.
These devices provide a greater level of assist and output
response than an intra-aortic balloon pump. Devices may
be small, impeller-type systems such as the Impella series
of recovery devices (Abiomed) designed for very shortterm use, or the TandemHeart (Cardiac Assist), which is
an extracorporeal centrifugal flow pump. This device may
also be used for relatively brief periods of time (Figs 57.3
and 57.4).
Long-term assist devices are now predominantly axial
flow devices or centrifugal pumps. These devices may be
directly implanted in individuals who are severely ill or in
individuals who are chronically severely debilitated and
require emergent, urgent, or elective placement of the
device. The most detailed data on the use of these devices
comes from the Interagency Registry for Mechanically
Assisted Circulatory Support (INTERMACS).5 INTERMACS
has defined seven different profiles of patients based upon
clinical severity and is tracking these patients over time
to determine outcomes. Ongoing evaluation of patients
having these devices placed will help determine best
practices. The basic INTERMACS profiles are shown in
Table 57.1.
As assist devices have evolved, so have indications.
Bridge to recovery is predominantly confined to acute
devices, with the larger devices showing only very
1225
Figs 57.4A and B: (A) The TandemHeart device. An inflow catheter is inserted in a femoral vein and moved up to the
right atrium and then across to the left atrium. Blood is removed from the left atrium outside to the pump and then via
the outflow cannula ejected into the central aorta; (B) Closer look at ideal position of the inflow cannula. Note there are
14 side holes and 1 end-hole.
Source: Used with permission from Cardiac Assist, Inc.
Table 57.1: Classification of Levels of Severity of Patients who are Potential Candidates for a Ventricular Assist Device: The
Interagency Registry for Mechanical Assisted Circulatory Support (INTERMACS)
Profile Description
16
38
Progressive Decline
28
Staple Inotropic Dependent Achieved stable blood pressure, organ perfusion, and nutrition but unable to be weaned from inotropic or other temporary
mechanical support
12
Resting Symptoms
Exercise Intolerant
Exertion Limited
1226
Table 57.2: Frequency of Ventricular Assist Device Implantation by Device Strategy: The INTERMACS Registry
Implant Year
2007
2009
2011
Bridge to Transplant
147 (42%)
505 (49%)
424 (22%)
Bridge to Candidacy
134 (38%)
432 (42%)
695 (37%)
Destination Therapy
48 (14%)
59 (6%)
742 (39%)
Bridge to Recovery
14 (4%)
13 (1%)
17 (1%)
Other
8 (2%)
11 (1%)
20 (1%)
351
1,020
1,898
Total Implants
REVERSE REMODELING
Placement of a LVAD generally causes significant reverse
remodeling. While it was hoped initially that substantial
reverse remodeling would include both volumetric
reduction and functional improvement, to the point that the
LVAD might be removed, later studies in wide varieties of
individuals have shown that the ability to explant an LVAD
is relatively uncommon.6 In an analysis of neurohormonal
blood levels in patients with LVADs, some studies have
shown a decline in levels of endothelin-1 and B-type
natriuretic peptide associated with some improvement
in function. Analysis of myocardium taken from the core
sample at the time of LVAD placement and then at the
time of either explantation or transplantation has shown
improvements in cardiac myocytes. In particular, there has
been regression of cardiac cell hypertrophy and reduction
in overall size. In addition, there has been evidence of a
reduction in total collagen back toward control levels.
Replacement fibrosis for dead myocytes generally does
not revert, but the interstitium may change favorably
with unloading from the LVAD. Unfortunately, these
positive changes in matrix and myocytes have generally
not been translated into enough improvement to allow
explantation.7,8
TYPES OF DEVICES
Short-Term Circulatory Support
Short-term devices are indicated for patients with acute
cardiogenic shock or postcardiotomy shock, which could
involve the left ventricle, right ventricle, or both. In some
circumstances, these devices may be placed prior to
performing a high-risk percutaneous intervention.
1227
Abiomed AB5000
TandemHeart System
The TandemHeart is an extracorporeal centrifugal pump.
The inflow catheter is inserted via a femoral vein, up the
vena cava and into the right atrium, and then via transseptal puncture placed in the left atrium. The device
removes oxygenated blood from the left atrium, out of the
body to the external pump, which then returns this blood via
a femoral artery into the central aorta. The inflow catheter
has 14 side holes and an end hole. Transesophageal
echocardiography is used by some operators to help
assist in trans-septal puncture and placement of the
inflow catheter. The TandemHeart has been compared
to intra-aortic balloon pulsation in patients presenting
with cardiogenic shock. The hemodynamic effects of the
TandemHeart appear to be superior (see Figs. 57.4A and B).
However, overall mortality was not reduced.10
1228
Jarvik-2000 FlowMaker
The pump is different from other axial flow devices in that
there is no inflow cannula. The pump is directly implanted
in the apex of the left ventricle but can also be used in the
right ventricle. This pump is also relatively small at 90 g
(Fig. 57.5).
HeartAssist System
This is an axial flow pump that is relatively small, being
only 95 g in weight. The device rotates between 7,500 and
1229
Name
Manufacturer
Pump Type
Inflow/Outflow
Anatomic Site
Speed Range
Current Usage
axial/
catheter based
LV/aorta RV/PA
2550 K (2.5)
1030 K (5)
Short-term up
to 6 hours
LA/femoral artery
37.5 K
Short-term up
to 30 days
TandemHeart
Pulsatile/extracorporeal
LV apex/aorta RA/
PA
Pulsatile
Short-term
support up to
30 days
CentriMag
Thoratec Corp.
(Pleasanton, CA)
Centrifugal/extracorporeal
LV apex/aorta RA/
PA BiVAD, ECMO
05.5 K
Up to 30 days
support
Incor
Axial/intracorporeal
LV apex/aorta
7.5 K
Long-term
support
HeartMate II
Axial/intracorporeal
LV apex/aorta
615 K
Long-term
support
HeartAssist 5
MicroMed Technology
(Houston, TX)
Axial/intracorporeal
LV apex/aorta
7.512.5 K
Long-term
support
Jarvik 2000
Axial/intracorporeal
LV apex/aorta RV/
PA BiVAD
812 K
Long-term
support
HeartWare HVAD
HeartWare International
(Framingham, MA)
Centrifugal/intrapericardial LV apex/aorta
1.84 K
Long-term
support
DuraHeart
Centrifugal/intrapericardial LV apex/aorta
1.22.4 K
Long-term
support
Levacor
Centrifugal/intracorporeal
LV apex/aorta
13 K
Long-term
support
EvaHeart
Centrifugal/intracorporeal
LV apex/aorta
12.8 K
Long-term
support
Long-Term Support
(LV: Left ventricle, PA: Pulmonary artery, BiVAD: Biventricular assist device, ECMO: Extracorporeal membrane oxygenator; RA: Right
Atrium).
PREOPERATIVE ECHOCARDIOGRAPHIC
EVALUATION
Once clinical criteria have been established that
suggest a ventricular assist device may be necessary, a
Left Ventricle
The left ventricle should be carefully examined with full
long-axis and short-axis imaging. Particular attention
1230
Figs 57.6A and B: Two examples of apical thrombi. (A) Large thrombus is easily seen in the apical region of the ventricle; (B) This
thrombus was not apparent with routine imaging but was much better characterized with contrast.
Fig. 57.7: Severe dilated cardiomyopathy baseline study. Dimensions serve as a frame of reference for serial follow-up after placement of the device. (Ao: Aorta; LA: Left atrium; LV: Left ventricle;
RV: Right ventricle).
1231
Fig. 57.9: Example of a patient with severe mitral regurgitation due to papillary muscle dysfunction. In most cases, severe
mitral insufficiency is not a contraindication for left ventricular assist
device (LVAD) placement. In many patients, decompression of the
left ventricle (LV) improves coaptation. (RV: Right ventricle).
Left Atrium
Mitral Valve
The mitral valve morphology should be evaluated.
LVADs are contraindicated in patients with significant
mitral stenosis. This is readily evaluated by Doppler
flows across the mitral valve. The motion of the leaflets
should be determined and the severity of mitral regurgitation evaluated (Fig. 57.9). In most circumstances,
mitral regurgitation will be expected to improve with
decompression of the left ventricle after placement of the
LVAD. Appropriate quantification of the severity of mitral
insufficiency preoperatively helps establish a baseline to
evaluate the effect of the LVAD after it is implanted.
Pericardium
The pericardium should be assessed to determine if any
significant effusion is present or if there is any evidence
that suggests the presence of constriction.
Aortic Valve
Aortic valve anatomy and function is particularly important
if the temporary impeller device is to be utilized. This
device works well if the aortic valve is normal, but presence
Atrial Septum
The atrial septum is an important structure, particularly
since relative filling pressures may change dramatically
1232
Figs 57.11A and B: (A) Dilated cardiomyopathy with small secundum atrial septal defect (ASD) with shunt shown in orange going
left to right. This was not detected in the past and may be due to stretching open of the foramen caused by chamber enlargement;
(B) Different patient with heart failure and a larger atrial septal defect. Note large orange left to right shunt jet. Both require closure during
left ventricular assist device (LVAD) surgery.
Aorta
Examine as much of the aorta as possible. Off-axis views
may be of value to show as much of the ascending aorta as
Tricuspid Valve
The tricuspid valve can pose particular challenges in the
perioperative period. Careful evaluation of the severity
of tricuspid valve insufficiency is essential from all
standard views. Characterization of leaflet motion and
morphology, along with measurement of the diameter of
the annulus, can be of considerable help to the surgeon in
determining whether or not tricuspid valve repair is indicated during the procedure (Figs 57.12A to D and Movie
clips 57.4 and 57.5).1517
Pulmonic Valve
The pulmonic valve should be evaluated to exclude
any unknown stenosis and also carefully evaluated to
determine if there is any significant pulmonic valve insufficiency. This is particularly important in patients who may
have underlying congenital disease. Substantial amounts
of pulmonic valve insufficiency could contribute to right
ventricular overload following placement of the LVAD.
1233
Figs 57.12A to D: Tricuspid valve. (A) Valve morphology shows evidence of mild leaflet thickening and reduced excursion due to right
ventricle (RV) enlargement and dysfunction; (B) Severe valve insufficiency is present; (C) Coaptation is severely compromised, leaving
a visible regurgitant orifice (arrow); (D) The annulus is markedly dilated. This patient had tricuspid valve repair when the left ventricular
assist device (LVAD) was placed. (LV: Left ventricle; RA: Right atrium).
Right Ventricle
Perhaps the most difficult and perplexing pre-LVAD
evaluation is that of the right ventricle. The concern is
1234
IMMEDIATE POSTSURGICAL
EVALUATION
Immediate postoperative evaluation is generally
performed by the cardiac anesthesiologist after the patient
comes off the pump.29 The immediate surgical results of
the LVAD placement should be evaluated, along with the
response of the ventricles and valves.
Before the assist device is activated, an immediate
evaluation for residual air bubbles is undertaken in the
ventricular chambers, ascending aorta, and cannulas.
Activation of the LVAD should begin appropriate
unloading and should result in a slight change in
interventricular and intra-atrial septum contours to
the left. Lack of effective decompression will result in
substantial rightward deviation of the septum, indicating
suboptimal support from the assist device or inadequate
settings to unload the left ventricle. This may require rapid
assessment of LVAD function and the device cannulas.
The opposite can also occur. Extreme leftward shift of the
septum suggests the possibility of an excessively high pump
speed that may be unloading the left side too vigorously. It
could also be caused by right ventricular dysfunction; thus,
right ventricular function should be assessed immediately
along with tricuspid valve performance, particularly for
severity of tricuspid regurgitation. An immediate concern
1235
Table 57.4: Evaluation of the Right Ventricle: Factors that May Impact Response to Placement of Left Ventricular Assist Device
Factors
Comment
Tricuspid Valve
Severity of regurgitation (Fig. 57.12)
Values < 20% are associated with increased risk of post LVAD RV
dysfunction.24
RVEDD/LVEDD ratio (by transesophageal echocardiography) Ratio > 0.72 associated with adverse outcome.17,26
Right-sided Hemodynamics
Central vanous pressure (CVP)
Points
LVEDD
> 78 mm
7078 mm
< 70 mm
LVEF
< 19%
1933%
> 33%
Sensitivity
Specificity
3 points
88.6%
47.4%
4 points
71.4%
67.1%
5 points
42.9%
80.3%
LAD/LVEDD
< 0.63
0.630.68
> 0.68
(CVP: Central venous pressure; LAD: Left atrial dimension; LVEDD: Left ventricular end-diastolic dimension; LVAD: Left ventricular assist device; RV: Right ventricle; RVEDD: Right ventricular end-diastolic dimension; TAPSE: Tricuspid annular plane systolic
excursion).
1236
Table 57.5: Points of Emphasis for the Pre-Left Ventricular Assist Device Echocardiogram
Left Ventricle: Size, geometric shape, systolic function, diastolic function, and filling pressure
Left Ventricular Apex: Shape, trabeculae, and thrombus
Aortic Valve: Leaflet motion, morphology, severity of valve insufficiency, and presence of stenosis
Mitral Valve: Presence of stenosis, motion of leaflets, and severity of valve insufficiency
Tricuspid Valve: Annular dimension and severity of valve insufficiency
Atrial Septum: Patent foramen ovale and atrial septal defect
Pulmonic Valve: Severity of valve insufficiency
Inferior Vena Cava: Estimate of central venous pressure
Aorta: Ascending aortic size and atherosclerotic plaque
Pericardium: Effusion or constrictive changes
Right Ventricle: Size, geometric shape, and systolic function
Figs 57.13A to C: Two examples of calculation of tricuspid regurgitation duration. In (A) the duration is quite prolonged, giving a rate
corrected value of 631 ms. This puts the patient in a prognostically
favorable group; In (B) the rate corrected value is 458 ms, putting
the patient in the prognostically less favorable group even though
the severity of the valve regurgitation was moderate; (C) and
right ventricular systolic pressures were only mildly increased.
(LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right
ventricle).
1237
1238
Figs 57.16A and B: (A) Transesophageal echo high esophageal view showing location of the outflow cannula in the ascending aorta
with color flow signal. The arrows show aorta landmarks for reference of location; (B) Continuous wave Doppler of outflow showing
pulsatile flow preservation. (AV: Aortic valve annular plane; ST: Sinotubular junction).
Fig. 57.17: Specialized views obtained to help visualize structures in the patient with a left ventricular assist device (LVAD).
Source: Reproduced with permission from Elsevier.
1239
1240
Figs 57.18A and B: Example of pre- and post-left ventricular assist device (LVAD) dimensions taken at 3 months following LVAD placement. There has been significant decompression of the left ventricle (LV), with a 25% decline in LV end diastolic dimension.
Figs 57.19A and B: (A) Example of off-axis apical view showing the inflow cannula having relatively low inflow velocity (B).
COMPLICATIONS OF LEFT
VENTRICULAR ASSIST DEVICES
Evidence of Left Ventricular Over-Filling
Normally, the LVAD is expected to decompress the left
ventricle, reduce its size, and maintain the changes
1241
1242
Figs 57.22A to D: (A) Example by M-mode of intermittent opening of the aortic valve (AV); (B) Typical central jet that can develop after
left ventricular assist device (LVAD) placement; (C) M-mode with color of same patient as (B) shows continuous nature of insufficiency
flow; (D) Continuous wave (CW) Doppler from the apical view in the same patient. Note that even though the left ventricle (LV) contracts,
it does not generate enough force to open the valve.
1243
Figs 57.23A and B: (A) Mitral flow prior to placement of left ventricular assist device (LVAD) when the patient was in class IIIb heart failure; In
(B) the LVAD has been in place for 2 months. Note the improvement in deceleration even though the E/A ratio remains elevated.
Table 57.6: Early Serial Changes in Ventricular Size and Function after Placement of a Left Ventricular Assist Device
Pre-Op
Three Months
Six Months
LVEDD (mm)
70
61
60
LVESD (mm)
62
54
54
17
18
16
RVEDD (mm)
35
34
34
RV Function
1.5
1.6
1.4
12
46
26
27
2.5
1.6
1.5
Mitral DT (ms)
146
200
195
E/e Ratio
13.3
11.8
11.4
Diastolic Grade*
2.5
2.0
2.1
LA Volume (mL)
95
73
65
*For both RV function and severity of diastolic dysfunction, the score was: 0 = normal, 1 = mildly abnormal, 2 = moderately abnormal,
3 = severely abnormal.
(CVP: Central venous pressure; DT: Deceleration time; EDD: End-diastolic dimension; EF: Ejection fraction; ESD: End-systolic
dimension; LA: Left atrium; LV: Left ventricle; RV: Right ventricle; SP: Systolic pressure).
Source: Adapted from reference 28.
1244
Figs 57.24A and B: Patient who suffered sudden power failure of his left ventricular assist device (LVAD). (A) There was a significant
change in the aortic valve M-mode that began fully opening in each beat; (B) The flow signal at the inflow cannula showed evidence of
weak forward flow, upper arrow, and regurgitant flow (lower arrow). The device did not clot in the 6 hours before surgery. (AO: Aorta;
LA: Left atrium; LV: Left ventricle; RV: Right ventricle).
Figs 57.25A and B: This patient had normal inflow cannula velocity (A). Two months later, there is a change in the inflow pattern consistent with obstruction. In this case, velocity peaks near end-systole (B). Peak velocity has increased from about 40 cm/s to almost
200 cm/s.
A
Figs 57.26A and B
1245
Increased LVEDD
Septal contour shift rightward
Spontaneous contrast in LV, LA
Increase in mitral regurgitation
Change in mitral inflow signal, E/e suggesting increased filling pressure
Increased opening of aortic valve to every beat
Atrial septal contour shifted rightward
Cause
Pump readout changes
Specific echocardiographic
findings
Inflow obstruction
Reduced flow
Reduced flow
Increased power
consumption
Spikes in power
Apical thrombus
Apical vegetation
(LA: Left atrium; LV: Left ventricle; LVAD: Left ventricular assist device; LVEDD: Left ventricular end-diastolic dimension).
1246
Figs 57.27A to C: The left ventricle (LV) cavity is small and the gap
between the cannula and septum satisfactory in systole (A)
but very small toward end-diastole (B). The inflow signal shows
increased flow velocity at end-diastole corresponding to the
findings (C). Diuretics were stopped and the left ventricular assist device (LVAD) speed reduced to 8,800 rpm. Inflow velocity
normalized.
1247
Potential causes
Pump readouts
Reduced output
Normal power usage
Occasional set down episodes due to inflow cannula obstruction
1248
1249
EXPLANTATION
A small group of patients show recovery of function
sufficient to allow consideration for explantation.6 A few
disease processes are inherently self limited and have
an optimistic outlook for recovery. However, in cases of
true dilated cardiomyopathy, the chance of recovery is
very low. For instance, in one series of 1,108 patients,
20 (1.8%) had the HeartMate II explanted due to recovery
of left ventricular function. This trial and others have
also suggested that a nonischemic etiology is more likely
to recover. Of the 1,108 patients cited above, 531 were
nonischemic; they had a recovery rate of 3.4%. A group of
578 of the patients were ischemic; they had a recovery rate
of only 0.3%. Age also appears to be a significant factor.
Patients < 40 years of age have a higher rate of recovery,
and patients who have a shorter duration of heart failure
symptoms tend to have a higher rate of recovery.41,42 These
general findings have also been consistently found in
trials evaluating medical management of dilated cardiomyopathy.
Echocardiography plays a central role in identifying
patients who may be considered for explantation. Weaning
protocols were more easily performed with pulsatile
pumps since they could be temporarily slowed down to
a very low rate while patients were carefully monitored.
The transition to continuous flow devices has made
weaning more difficult. Some devices can be shut down
temporarily; others can only be partially slowed down.
Specific weaning protocols are beyond the scope of this
chapter and tend to be institution-specific.43 It should be
1250
Table 57.9: Optimization of Left Ventricular Assist Device Performance: Echocardiographic Measurements Indicating a Favorable
Response
PERCUTANEOUS CONTINUOUS
FLOW DEVICES
Impella Device
This device is devised for short-term implantation. The
device is inserted generally through a large femoral
artery and retrogradely brought into position across
the aortic valve so that the distal portion of the device
suctions blood out of the left ventricle, across the
valve, and deposits the blood in the ascending aorta
(Fig. 57.3). Since this is a retrograde percutaneous device,
several different considerations before placement of the
TandemHeart
The TandemHeart (see Fig. 57.4) typically utilizes
a femoral vein and femoral artery with an external
pump.10 These devices are frequently placed using
1251
1252
C
echocardiographic imaging support, commonly transesophageal echocardiography. Preprocedure TEE
screening is of considerable value to evaluate not only
issues of ventricular and valvular function but also to
carefully evaluate the left and right atrial chambers for
any unusual anatomical abnormalities or thrombi that
would complicate placement of the large-bore catheter
moved retrograde up through the vena cava and then
trans-septally across the atrial septum. TEE is frequently
used for guidance of the trans-septal puncture, and
once the device is placed for correct positioning of the
retrograde catheter in the left atrium. Color Doppler can
assist in detecting flow since the device has multiple
holes at the end of its inflow catheter (see Figs 57.4A
and B). As with other assist devices, echocardiography can
serially evaluate the effect of the device. Also, if the device
appears to be working improperly, echocardiographic
imaging, particularly to evaluate the position of the inflow
cannula, may be of great value (Figs 57.30A to C and Movie
clips 57.16 to 57.17, 57.18). In some circumstances, this
cannula could migrate either further inward or backward
across the atrial septum and become malpositioned. These
catheters also can form clots and sometimes become
obstructed, which echocardiography can identify.
REFERENCES
1. Liotta D, Crawford ES, Cooley DA, et al. Prolonged partial
left ventricular bypass by means of an intrathoracic pump
implanted in the left chest. Trans Am Soc Artif Intern
Organs. 1962;8:909.
2. DeBakey ME. Left ventricular bypass pump for cardiac
assistance. Clinical experience. Am J Cardiol. 1971;27(1):
311.
3. Rose EA, Gelijns AC, Moskowitz AJ, et al.; Randomized
Evaluation of Mechanical Assistance for the Treatment of
4.
5.
6.
7.
8.
9.
10.
11.
12.
Congestive Heart Failure (REMATCH) Study Group. Longterm use of a left ventricular assist device for end-stage
heart failure. N Engl J Med. 2001;345(20):143543.
Nose Y, Motomra T, Miyamoto H. History of Mechanical
Circulatory Support. In: Joyce DL, Joyce LD, Loebe M,
editors. Mechanical Circulatory Support. New York, NY:
McGraw Hill Medical; 2012:312.
Interagency Registry for Mechanically Assisted Circulatory
Support. Quarterly Statistical Report 2012, 3rd Quarter.
Available from www.intermacs.org. 2012; Accessed January
2013.
Mancini DM, Beniaminovitz A, Levin H, et al. Low incidence
of myocardial recovery after left ventricular assist device
implantation in patients with chronic heart failure.
Circulation. 1998;98(22):23839.
Bruckner BA, Stetson SJ, Farmer JA, et al. The implications
for cardiac recovery of left ventricular assist device
support on myocardial collagen content. Am J Surg. 2000;
180(6):498501; discussion 501.
Bruckner BA, Stetson SJ, Perez-Verdia A, et al. Regression of
fibrosis and hypertrophy in failing myocardium following
mechanical circulatory support. J Heart Lung Transplant.
2001;20(4):45764.
Abiomed, product description. Available at www.abiomed.
com. 2013; Accessed January 2013.
Burkhoff D, Cohen H, Brunckhorst C, et al. A randomized
multicenter clinical study to evaluate the safety and
efficacy of the TandemHeart percutaneous ventricular
assist device versus conventional therapy with intraaortic
balloon pumping for treatment of cardiogenic shock. Am
Heart J. 2006;152(3):469.e1469.e8.
Marks J, Macedo M, Dasse K. Levitronix CentriMag and
PediVAS Systems: Applications and clinical results. In:
Joyce D, Joyce L, Loebe M, editors. Mechanical Circulatory
Support: Principles and Applications. New York, NY:
McGraw Hill Medical; 2012:1605.
Nishinaka T, Miller PJ, Bearnson GB, et al. EVAHEART left
ventricular assist system. In: Joyce D, Joyce L, Loebe M,
editors. Mechanical Circulatory Support: Principles and
Applications. New York, NY: McGraw Hill Medical; 2012:
23841.
1253
1254
CHAPTER 58
Echocardiographic Assessment of
Left Atrial Function
Utpal N Sagar, Hirohiko Motoki, Allan L Klein
Snapshot
Anatomy
Physiology
INTRODUCTION
As the role of echocardiography has evolved to assess
hemodynamic status and diastolic function in addition to
characterizing two-dimensional structure and function,
there has been an emphasis on improving echocardi
ographic assessment of the left atrium (LA). This has
become of increasing importance as left atrial volume
and function have been described as strong predictors
of major adverse cardiovascular events. In this chapter,
we will review the structure and multifaceted function of
the LA, the physiology of the LA, modalities of functional
assessment, and review left atrial function in the context of
various cardiovascular disease states.
ANATOMY
The LA is the most posteriorly situated chamber of the
heart and is oriented superiorly and to the left of the right
atrium. The pulmonary veins normally drain into the
posterior aspect of the LA. These vessels are covered by the
visceral or inner layer of pericardium. The serous layer is
fused with the outer fibrous pericardium.1
The LA may be divided into different regions,
including the left atrial appendage which is small, tubular,
Functional Assessment
1256
Fig. 58.1: Left atrial (LA) phasic functions and their relationship
with the cardiac cycle. (TMF: Transmitral flow; PVF: Pulmonary
vein flow, ECG: Electrocardiogram). Note the changes in left atrial
volumes through the various phases of left atrial function.
PHYSIOLOGY
Although the role of the LA could be simply described as a
chamber that receives oxygenated blood, its function is far
more complex. Below, we will discuss the phasic function
of the LA and the factors that affect its function.
Physiological Effects on
Left Atrial Function
It follows that the previously described left atrial function
do not occur in isolation and are related to left ventricular
compliance. Abnormalities in left ventricular, valvular, or
atrial disease are often reflected as an increase in left atrial
filling pressures, which may be observed as enlargement
of left atrial size.4 Hence, LA afterload increases as
left ventricular (LV) filling pressures increase and as
LV diastolic dysfunction worsens. This increase in LA
afterload and LA volume results initially in an increase in
LA size and an improvement in LA function. However, LA
contractility declines once a threshold has been reached,
similar to the FrankStarling curve of the LV.2 Various
examples of the relationship between LA and LV size, and
functional assessment will be discussed in the left atrial
pathophysiology section of this chapter.
FUNCTIONAL ASSESSMENT
We have already described the significant interplay
between LA and LV function, such that events during
each phase of LA phasic function are affected by factors
from both the LA and LV. However, despite considerable
investigation, the magnitude and relative importance
of the atrial contribution to LV filling and cardiac output
remain controversial, and provide a motivation for a more
complete evaluation of the atrial cycle.
Recent advances in catheter ablation for the treat
ment of atrial fibrillation (AF), in dual- and threechamber pacemakers that maintain atrioventricular and
biventricular synchrony, and in the pathological and
clinical relevance of chamber-specific structural, elec
trical, and ionic remodeling have increased the interest
in accurately imaging the LA structure and function.
With respect to the assessment of the LA function, twodimensional echocardiography (2DE), three-dimensional
echocardiography (3DE), Doppler echocardiography,
and speckle tracking echocardiography have distinctly
different strength and weaknesses, and are complementary
in specific clinical scenarios. In this section, we discuss
the role of each modality to assess LA function with
an emphasis on the relative merits of newer imaging
techniques and how these may be applied in the various
clinical settings.
Volume
Left atrial size has been compared to the hemoglobin A1c
in diabetes as a measure of the average effect of LV filling
1257
Spectral Doppler
Pulmonary vein flow, transmitral flow, and mitral annular
velocity are routinely measured. These parameters are
determined by LA function, as well as LV systolic and
diastolic performance. Peak velocity of mitral A-wave
indicates LA mechanical function, although it is also
affected by heart rate and loading conditions.28 Peak
mitral A velocity has been shown to be associated with
1258
Figs 58.3A to E: Various methods of echocardiographic determination of left atrial size. Figure A demonstrates M-mode through the
left atrium (LA), Figures B and C demonstrate volume measurements of the LA using the biplane method of discs (modified Simpsons
rule) from apical four-chamber and two-chamber views, respectively. Figures D and E demonstrate the measurement of LA volume from
arealength method using the images from apical four-chamber and two-chamber views.
Fig. 58.4: Multiplanar imaging of the left atrium, with three-dimensional rendering and determination of the left atrial volume.
1259
Hypertension
Speckle Tracking
Measures of myocardial deformation have been
increasingly adapted to study LA mechanics. Both echo
cardiographic methods of measuring strain and SR, 2D
speckle tracking imaging, and color TDI have been adapted
to measuring LA deformation. Speckle tracking calculates
strain by tracking tissue deformation via characteristic
myocardial speckles frame-by-frame, with SR given by the
rate of such deformation (Fig. 58.5). Color TDI generates
a spatial map of myocardial velocities, from which SR of
the region of interest is derived, with strain calculated by
integrating the SR data.
The advantage of analyzing LA mechanics with strain
and SR imaging is the information that can be obtained
about each component of LA phasic function. One could
use this method to resolve the exact change in LA phasic
function with different disease states and investigate the
effect after treatment.
Thus, deformation-based parameters of LA reservoir
function could provide the prognostic information in the
population at risk for adverse cardiovascular events.
Atrial Fibrillation
Impairments in LA structure and function may commonly
lead to AF. Various echocardiographic modalities have
been studied to evaluate the degree of LA fibrosis that
has been associated with AF. Atrial conduction time
as measured by TDI has been shown to predict the
development of AF in the general population when
prolonged greater than 190 ms.35 Recently, percutaneous
catheter ablation has been used more frequently to
successfully manage symptomatic and medically refractory
AF. In these patients, the best predictors for maintenance
of sinus rhythm following ablation were parameters of LA
reservoir function, determined by SR imaging.36,37 Studies
have shown that LA reservoir function has been related to
LA structural remodeling and fibrosis of the atrial wall.38,39
Following conversion to sinus rhythm, LA volume and
function may be monitored, with those patients who have
greater degrees of dysfunction being selected for further
therapy, such as antiarrhythmics.40
Direct current cardioversion is a widely used treatment
modality for both emergent and elective manage
ment of AF. However, there is significant risk of stroke
and thromboembolism associated with direct current
cardioversion.4144 Classically, this was thought to be
due to pre-existing left atrial thrombus or continuous
or prominent spontaneous echo contrast. Earlier, we
described the location and anatomy of the left atrial
appendage. Transesophageal echocardiography (TEE) is
1260
Cardiomyopathies
Assessment of LA function may help to diagnose, to
differentiate, and to guide therapy of various cardio
myopathies. For example, patients with hypertrophic
cardiomyopathy have been shown to have decreased
LA longitudinal function in addition to decreased LA
reservoir function. This may be used to differentiate
hypertrophic cardiomyopathy from other forms of left
ventricular hypertrophy.49,50 Figures 58.7A to E show an
Figs 58.6A to C: The spectrum of left atrial appendage pathology. Figure A shows the presence of left atrial spontaneous echocontrast,
or smoke; Figure B demonstrates prominent, persistent spontaneous echocontrast in the left atrium and left atrial appendage (LAA),
consistent with sludge; Figure C shows the presence of a minimally mobile echodensity within the left atrial appendage, consistent
with thrombus (arrow).
Figs 58.7A to E: Patient with hypertrophic obstructive cardiomyopathy (HOCM), as seen in Figure A. Figures B and C demonstrate
severe left atrial enlargement. Mitral inflow shows decreased A-wave (Figure D) and tissue Doppler image (Figure E) shows moderate
to severe decreased atrial contraction with decreased a' velocity. (LA: Left atrium; LV: Left ventricle).
1261
Figs 58.8A to D: Echocardiogram of a patient with cardiac amyloidosis. The apical four-chamber view in Figure A demonstrates the
degree of left atrial enlargement; Figure B shows the degree of increased left ventricular wall thickness and the characteristic echotexture of a patient with cardiac amyloidosis. The transmitral flow demonstrating restrictive diastolic pattern with small A-wave is seen in
Figure C. Tissue Doppler in Figure D shows severely depressed a' velocity, consistent with restrictive cardiomyopathy. (LA: Left atrium;
LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
REFERENCES
1. Ho SY, Cabrera JA, Sanchez-Quintana D. Left atrial anatomy
revisited. Circ Arrhythm Electrophysiol. 2012;5:2208.
2. Blume GG, McLeod CJ, Barnes ME, et al. Left atrial function:
physiology, assessment, and clinical implications. Eur
J Echocardiogr. 2011;12:42130.
3. To AC, Flamm SD, Marwick TH, et al. Clinical utility of
multimodality LA imaging: assessment of size, function,
and structure. JACC Cardiovasc Imaging. 2011;4:78898.
4. Leung DY, Boyd A, Ng AA, et al. Echocardi
ographic
evaluation of left atrial size and function: current under
standing, pathophysiologic correlates, and prognostic
implications. Am Heart J. 2008;156:105664.
5. Abbas AE, Fortuin FD, Schiller NB, et al. A simple method
for noninvasive estimation of pulmonary vascular resis
tance. J Am Coll Cardiol. 2003;41:10217.
6. Douglas PS. The left atrium: a biomarker of chronic
diastolic dysfunction and cardiovascular disease risk. J Am
Coll Cardiol. 2003;42:12067.
1262
38.
39.
40.
41.
42.
43.
44.
1263
CHAPTER 59
The Use of Echocardiography to
Assess Cardiac Hemodynamics and
Guide Therapy
Roy Beigel, Robert J Siegel
Snapshot
Le Atrial Pressure
INTRODUCTION
Accurate noninvasive hemodynamic assessment has the
potential to greatly improve patient management with
regard to volume status, pharmacological treatment,
and clinical outcomes. Several studies have shown that
the time-honored physical examination has very
limited sensitivity and specificity for right atrial pressure
(RAP), pulmonary artery pressure (PAP), as well as
left atrial (LA) filling pressures. Since the 1970s, the
standard for hemodynamic assessment has been invasive
measurements made by pulmonary artery (PA) catheterization. However, use of the PA catheter has been subject
to criticism13 as it can increase patient morbidity.4,5
Doppler-echocardiographic measurements of right- and
left-sided filling pressures, pulmonary vascular resistance
(PVR), and cardiac output (CO) are possible to obtain in
most patients. Echocardiography can potentially provide
adequate alternative hemodynamic data, which are more
accurate than the physical examination without the risks
of invasive monitoring.
Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy
1265
Table 59.1: The Various Methods Utilized for the Echocardiographic Evaluation of Right Atrial Pressure (RAP)
Method
Criteria Used
VsVTI/(VsVTI + VdVTI)
< 55High RAP > 8 mm Hg
> 59 ms correlates to
RAP > 8 mm Hg
3D RA dimensions3D
transthoracic imaging of the RA
(3DE: Three-dimensional echocardiography; IVC: Inferior vena cava; RA: Right atrial; SVC: Superior vena cava; TDI: Tissue Doppler
imaging; VTI: Velocity time integral).
1266
Fig. 59.1: Imaging of the inferior vena cava (IVC, marked with
asterisk) using 2D echocardiography (top, and middle images),
and M-mode echocardiography (bottom images) from the
subcostal view. The left three images show respiratory variations
of the IVC in a patient with normal right atrial pressure (RAP). The
right three images show no respiratory variation of the IVC, which
is also dilated. This patient was found to have an elevated RAP.
Table 59.2: The Various Methods Utilized for the Echocardiographic Evaluation of Pulmonary Artery Pressure (PAP)
Method
Criteria Used
Systolic Pulmonary Artery Pressure (SPAP):
Using the TR jetSimplified
P = 4 V2 + RAP = SPAP
Bernoulli equation (Fig. 59.5)
Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy
1267
Contd....
Method
Criteria Used
Diastolic Pulmonary Artery Pressure (DPAP):
Using the pulmonary
P = 4 V2 + RAP = DPAP
regurgitation (PR)
jetSimplified Bernoulli
equation (Fig. 59.9)
TR velocity at time of
pulmonic valve
openingSimplified
Bernoulli equation
P = 4 V2 + RAP = DPAP
PVR:
Formula using surrogates for
the transpulmonary pressure
gradient and transpulmonary
flow
PVR (WU) = 10 TR
velocity/RVOT VTI + 0.16
(PH: Pulmonary hypertension; PVR: Pulmonary vascular resistance; RAP: Right atrial pressure; RVOT: Right ventricular outflow tract;
TR: Tricuspid regurgitation; VTI: Velocity time integral).
1268
Figs 59.2A and B: Evaluation of the central venous flow pattern using pulsed wave Doppler imaging. (A) Systolic (S) and diastolic (D)
flow in the superior vena cava of an adult with a normal biphasic pattern. The S/D > 1 is supportive of normal right atrial pressure (RAP);
(B) S and D flow velocity in the hepatic vein in a patient with elevated RAP. There is diminished S, increased D, and an S/D < 1.
Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy
1269
Figs 59.3A and B: Doppler and tissue Doppler imaging (TDI) of the tricuspid valve. (A) Tricuspid inflow velocity Doppler recording
(E = 36.1 cm/s); (B) Tricuspid annular velocity, e , is 5.2 cm/s. In this patient, the E/e ratio is >6, which supports that the RAP is
>10 mm Hg.
Fig. 59.4: Measuring right atrial (RA) dimensions. 2D echocardiographic images from the apical view focusing on the right heart.
On the left, the measurements of the RA major and minor axis
diameters are estimated at 4.87 and 3.07 cm, respectively. On the
right, the RA area tracing, which is 13.7 cm2.
PULMONARY ARTERY
HEMODYNAMICS (TABLE 59.2)
Pulmonary arterial hemodynamics are important for
patient diagnosis management, and prognosis. Current
1270
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1271
Table 59.3: Causes for Inaccurate Estimation of Pulmonary Artery Pressure by Echocardiography
Cause
Resolution
Underestimation:
Variations in Doppler angle of interrogation
Multiple imaging planes to receive best Doppler signal, use of color Doppler
for optimal alignment with the regurgitant jet
Overestimation:
Overestimation of RAP
Contrast artifacts
Adequate use of contrast, and adequate analysis of the tricuspid Doppler signal
Figs 59.7A and B: Underestimation of systolic pulmonary artery pressure (SPAP). In this patient the estimated tricuspid regurgitation (TR)
gradient was 42 mm Hg. The estimated right atrial pressure (RAP) using inferior vena cava diameter of 2.78 cm and absence of respiratory collapse (bottom) was 15 mm Hg, giving an estimated SPAP of 57 mm Hg. However, the actual pulmonary artery catheter reading
for SPAP was 84 mm Hg. This underestimation was due to underestimation of the RAP, which was measured invasively as 40 mm Hg.
Figs 59.8A to C: Enhancing a poor tricuspid regurgitation (TR) Doppler signal (A) with saline (B) or a saline + blood mixture (C), which
gives even more enhanced results.
1272
Fig. 59.9: Evaluation of diastolic and mean pulmonary artery pressure (DPAP and MPAP) using Doppler imaging of the pulmonic
regurgitation (PR) jet. The maximal velocity (Vmax) is 281 cm/s
(asterisk), and the velocity at end diastole is 195 cm/s (arrow).
Using the Bernoulli equation: P = 4 V2, the maximal pressure
gradient of 32 mm Hg between the pulmonary artery (PA) and the
right ventricle (RV) during diastole is calculated and corresponds
with the MPAP. When added to the right atrial pressure (RAP), this
improves accuracy. The gradient at end diastole between the PA
and the RV is also calculated and is 15 mm Hg. When added to
the RAP, the DPAP is given.
Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy
1273
Table 59.4: The Various Methods Utilized for the Echocardiographic Evaluation of Left-Sided Filling Pressures (PCWP, LVEDP)
Method
Criteria Used
S > DNormal
S < Delevated LA pressure or normal in
young (<40 years) individuals.
Ar velocity < 35 cm/snormal
ArA-wave duration < 30 msnormal LA
pressure
Depth limitation
Marked cardiac enlargement
Left atrial motion artifact
Influenced by age (D dominant in young
individuals < 40 years)
Ar duration and velocity:
Not influenced by age
Applicable also in normal LVEF, MV
disease, and HCM
Hard to obtain good quality images for
interpretation
Contd...
1274
Contd...
Method
Criteria Used
,
,
E/ ratio < 8normal LV filling pressures
15 (for septal ) or > 12 (for lateral )
elevated LV filling pressures
Reduced with:
Aging
The presence of annular calcifications,
annular rings, prosthetic MV, MS
Increased with:
Moderate to severe MR
Constrictive pericarditis (lateral may be
less than septal in this situation)
May be affected by:
Preload in those with normal LVEF
LV relaxation
Systolic function
Lateral values higher than septal values
increased by:
Increased LA contractility
Decreased LVEDP
E/
Lateral ratio lower than septal
In patients with normal LVEF has low sensitivity and high specificity
In patients with mitral annular calcification, severe MR, or constrictive pericarditis
might not give an adequate estimate of
filling pressures.
Might not be valid for patients with acute
decompensated heart failure100
Propagation velocityVp
Vp > 50 cm/snormal
E/Vp > 2.5elevated PCWP > 15 mm Hg
(DT: Deceleration time; HCM: Hypertrophic cardiomyopathy; IVRT: Isovolumic relaxation time; LA: Left atrium; LAP: Left atrial pressure; LV: Left ventricle; LVEF: Left ventricular ejection fraction; PCWP: Pulmonary capillary wedge pressure, LVEDP: Left ventricular
end diastolic pressure; MR: Mitral regurgitation; MS: Mitral stenosis; MV: Mitral valve; RA: Right atrium).
Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy
1275
Figs 59.11A and B: Mitral valve inflow Doppler. Primary measurements include the peak early filling (E) and the late diastolic atrial
filling (A) velocities, deceleration time (yellow line) of the E-wave. (A) patient with a normal filling pattern, the E-wave is greater than the
A wave with a deceleration time of 206 ms (>160 ms); (B) Restrictive filling pattern, the E-wave is greater than the A-wave with a very
short deceleration time of 137 ms.
Table 59.5: Normal Values by Age Groups for Doppler and Tissue Doppler Variables for Estimation of Left Heart Hemodynamics
Measurement
Age Group
1620
2140
4160
> 60
E/A ratio
1.88 0.45
1.53 0.4
1.28 0.25
0.96 0.18
DT (ms)
142 19
166 14
181 19
200 29
IVRT (ms)
50 9
67 8
74 7
87 7
Septal (cm/s)
14.9 2.4
15.5 2.7
12.2 2.3
10.4 2.1
Lateral (cm/s)
20.6 3.8
19.8 2.9
16.1 2.3
12.9 3.5
Ar duration (ms)
66 39
96 33
112 15
113 30
Source: Adopted from Nagueh SF, Appleton CP, Gillebert TC, et al. Recommendations for the evaluation of left ventricular diastolic
function by echocardiography. J Am Soc Echocardiogr. 2009;22(2):10733.62
Table 59.6: Variables Affecting Mitral Inflow Parameters
Parameter
Variables
General parameters
E-wave
Aging
Preload
Alterations in left ventricular
(LV) relaxation
A-wave
Aging
LV compliance
Left atrial contractile function
E-wave deceleration
time (DT)
Aging
LV relaxation
LV diastolic pressure after mitral
valve opening
LV compliance
1276
Pulmonary venous flow also provides important information for the assessment of LV diastolic function and
LA filling pressure. In most patients, the best Doppler
recordings are obtained from the apical four-chamber
view with the pulmonary venous flow obtainable in
~approximately 90% of adult patients.81 As seen in Figures
59.12A and B, variables include the peak systolic velocity
(S), which is composed of two systolic components (S1,
S2), peak anterograde diastolic velocity (D), the S/D
ratio, and the duration and peak of the atrial reversal (Ar)
velocity waveform.
The S-wave is primarily influenced by changes in the
LA pressure, contraction, and relaxation (S1 component)
and by the stroke volume (SV) and pulse wave propagation
in the PA vasculature tree.82,83 The D-wave is influenced
by the same factors that influence the mitral E velocity.84
Ar duration and velocity are influenced by the LV late
diastolic pressure, atrial preload, and contractility.85 With
Figs 59.12A and B: Doppler of the pulmonary vein flow pattern from the apical four-chamber view. (A) normal Doppler study demonstrates
the peak systolic velocity (S), which is composed of two systolic components (S1, S2), peak anterograde diastolic velocity (D), and peak
of the atrial reversal (Ar) velocity waveform; (B) An examination from a patient with an elevated left atrial filling pressure demonstrating
a decrease in S and increase in D, resulting in an S/D ratio of <1.
Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy
1277
Figs 59.13A and B: Evaluation of left-sided filling pressures. (A) Mitral inflow velocities are measured (E, A), showing an E > A with a
pseudonormal filling pattern (deceleration time > 160 ms); (B) Lateral tissue Doppler imaging (TDI) that includes both systolic (S) and
diastolic velocities. The early diastolic velocity is expressed as , and the late diastolic velocity as . In this patient, the E/ is elevated
and equals89/4.66 = 19, implying elevated left-sided filling pressures.
1278
Table 59.7: Variables Used for the Evaluation of Filling Pressures in Those with Normal and with Decreased Ejection Fraction (EF)
Variable
E/A
NA
E/
< 8normal FP
812indeterminate
13elevated FP
Vp
> 50 cm/snormal
E/Vp > 2.5elevated FP >15 mm Hg
Ar-A
< 0 msnormal FP
029indeterminate
30 mselevated FP
< 0 msnormal FP
029indeterminate
30 mselevated FP
IVRT/TE-
> 2normal FP
< 2elevated FP
> 2normal FP
< 2elevated FP
< 30 mm Hgnormal FP
> 35 mm Hgelevated FP
< 30 mm Hgnormal FP
> 35 mm Hgelevated FP
> 1normal FP
< 1Elevated FP
Limited accuracy
(Avg: Average; EF: Ejection fraction; FP: Filling pressure; Lat: Lateral; NA: Not applicable; Sep: Septal).
Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy
1279
Fig. 59.14: Schematic approach for the evaluation of left-sided filling pressures using the various obtained echocardiographic parameters. (DT: Deceleration time; IVRT: Isovolumic relaxation time; LA: Left atrium; PV: Pulmonary vein; SPAP: Systolic pulmonary artery
pressure).
*Refers for averaged values from both septal and lateral tissue Doppler; for specific values see Table 7.
**See text for further details. Adopted from Nagueh SF, Appleton CP, Gillebert TC, et al. Recommendations for the evaluation of left
ventricular diastolic function by echocardiography. J Am Soc Echocardiogr. 2009;22(2):10733.62
1280
Fig. 59.15: Measurement of left ventricular end diastolic pressure (LVEDP) using the aortic regurgitation (AR) signal. Using
continuous wave Doppler through the aortic valve in diastole, the
end-diastolic AR velocity gives out the pressure gradient (white arrow) which equals to 4 3.42 = 46 mm Hg. Subtracting this value
from the systolic blood pressure gives an estimate of the left atrial
pressure during systole (V-wave). The LVEDP equals the diastolic
blood pressure4V2.
A WORD TO CONCLUDE
As noninvasive evaluation utilizes indirect indexes for
estimation of hemodynamic parameters, it is far superior
to bedside physical examination but it also has limitations.
However, in addition to providing hemodynamic data and
volume status, the Doppler data provides insight into the
pathophysiology of ventricular filling and emptying.
Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy
Fig. 59.17: Estimation of the left atrial (LA) pressure. On the top
image: color flow imaging demonstrating a shunt between the
right atrium (RA) and LA. On the bottom image: continuous wave
Doppler for estimation of the pressure gradient between the RA
and the LA (white arrow), which equals 4 2.742 = 30 mm Hg.
Adding this gradient to the estimated RAP gives the estimated
LA pressure.
REFERENCES
1. Gore JM, Goldberg RJ, Spodick DH, et al. A communitywide assessment of the use of pulmonary artery catheters in patients with acute myocardial infarction. Chest.
1987;92(4):7217.
2. Harvey S, Harrison DA, Singer M, et al. PAC-Man study
collaboration. Assessment of the clinical effectiveness of
pulmonary artery catheters in management of patients in
intensive care (PAC-Man): a randomised controlled trial.
Lancet. 2005;366(9484):4727.
3. Ivanov RI, Allen J, Sandham JD, et al. Pulmonary artery
catheterization: a narrative and systematic critique of
randomized controlled trials and recommendations for
the future. New Horiz. 1997;5(3):26876.
1281
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Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy
1283
1284
57. Abbas AE, Fortuin FD, Schiller NB, et al. A simple method
for noninvasive estimation of pulmonary vascular resistance. J Am Coll Cardiol. 2003;41(6):10217.
58. Rajagopalan N, Simon MA, Suffoletto MS, et al. Noninvasive
estimation of pulmonary vascular resistance in pulmonary
hypertension. Echocardiography. 2009;26(5):48994.
59. Scapellato F, Temporelli PL, Eleuteri E, et al. Accurate
noninvasive estimation of pulmonary vascular resistance
by Doppler echocardiography in patients with chronic failure heart failure. J Am Coll Cardiol. 2001;37(7):18139.
60. Haddad F, Zamanian R, Beraud AS, et al. A novel non-invasive method of estimating pulmonary vascular resistance
in patients with pulmonary arterial hypertension. J Am Soc
Echocardiogr. 2009;22(5):5239.
61. Gurudevan SV, Malouf PJ, Kahn AM, et al. Noninvasive
assessment of pulmonary vascular resistance using
Doppler tissue imaging of the tricuspid annulus. J Am Soc
Echocardiogr. 2007;20(10):116771.
62. Nagueh SF, Appleton CP, Gillebert TC, et al. Recommendations for the evaluation of left ventricular diastolic function by echocardiography. J Am Soc Echocardiogr. 2009;
22(2):10733.
63. Brutsaert DL, Sys SU, Gillebert TC. Diastolic failure:
pathophysiology and therapeutic implications. J Am Coll
Cardiol. 1993;22(1):31825.
64. Appleton CP, Hatle LK, Popp RL. Relation of transmitral
flow velocity patterns to left ventricular diastolic function:
new insights from a combined hemodynamic and Doppler
echocardiographic study. J Am Coll Cardiol. 1988;12(2):
42640.
65. Vanoverschelde JL, Raphael DA, Robert AR, et al. Left
ventricular filling in dilated cardiomyopathy: relation to
functional class and hemodynamics. J Am Coll Cardiol.
1990;15(6):128895.
66. Pinamonti B, Di Lenarda A, Sinagra G, et al. Restrictive
left ventricular filling pattern in dilated cardiomyopathy
assessed by Doppler echocardiography: clinical, echocardiographic and hemodynamic correlations and prognostic
implications. Heart Muscle Disease Study Group. J Am Coll
Cardiol. 1993;22(3):80815.
67. Giannuzzi P, Imparato A, Temporelli PL, et al. Dopplerderived mitral deceleration time of early filling as a
strong predictor of pulmonary capillary wedge pressure
in postinfarction patients with left ventricular systolic
dysfunction. J Am Coll Cardiol. 1994;23(7):163037.
68. Pozzoli M, Capomolla S, Pinna G, et al. Doppler echocardiography reliably predicts pulmonary artery wedge
pressure in patients with chronic heart failure with and
without mitral regurgitation. J Am Coll Cardiol. 1996;27(4):
88393.
69. Xie GY, Berk MR, Smith MD, et al. Prognostic value
of Doppler transmitral flow patterns in patients with
congestive heart failure. J Am Coll Cardiol. 1994;24(1):
1329.
70. Rihal CS, Nishimura RA, Hatle LK, et al. Systolic and
diastolic dysfunction in patients with clinical diagnosis
of dilated cardiomyopathy. Relation to symptoms and
prognosis. Circulation. 1994;90(6):27729.
71. Traversi E, Pozzoli M, Cioffi G, et al. Mitral flow velocity changes after 6 months of optimized therapy provide
important hemodynamic and prognostic information in
patients with chronic heart failure. Am Heart J. 1996;132
(4):80919.
72. Giannuzzi P, Temporelli PL, Bosimini E, et al. Independent
and incremental prognostic value of Doppler-derived mitral
deceleration time of early filling in both symptomatic and
asymptomatic patients with left ventricular dysfunction.
J Am Coll Cardiol. 1996;28(2):38390.
73. Hansen A, Haass M, Zugck C, et al. Prognostic value of
Doppler echocardiographic mitral inflow patterns: implications for risk stratification in patients with chronic
congestive heart failure. J Am Coll Cardiol. 2001;37(4):
104955.
74. Whalley GA, Doughty RN, Gamble GD, et al. Pseudonormal
mitral filling pattern predicts hospital re-admission in
patients with congestive heart failure. J Am Coll Cardiol.
2002;39(11):178795.
75. Bella JN, Palmieri V, Roman MJ, et al. Mitral ratio of peak
early to late diastolic filling velocity as a predictor of
mortality in middle-aged and elderly adults: the Strong
Heart Study. Circulation. 2002;105(16):192833.
76. Pinamonti B, Zecchin M, Di Lenarda A, et al. Persistence
of restrictive left ventricular filling pattern in dilated
cardiomyopathy: an ominous prognostic sign. J Am Coll
Cardiol. 1997;29(3):60412.
77. Temporelli PL, Corr U, Imparato A, et al. Reversible
restrictive left ventricular diastolic filling with optimized
oral therapy predicts a more favorable prognosis in
patients with chronic heart failure. J Am Coll Cardiol. 1998;
31(7):15917.
78. Yamamoto K, Nishimura RA, Chaliki HP, et al. Determination
of left ventricular filling pressure by Doppler echocardiography in patients with coronary artery disease: critical
role of left ventricular systolic function. J Am Coll Cardiol.
1997;30(7):181926.
79. Nishimura RA, Appleton CP, Redfield MM, et al. Noninvasive
Doppler echocardiographic evaluation of left ventricular
filling pressures in patients with cardiomyopathies: a simultaneous Doppler echocardiographic and cardiac catheterization study. J Am Coll Cardiol. 1996;28(5):122633.
80. Nagueh SF, Lakkis NM, Middleton KJ, et al. Doppler
estimation of left ventricular filling pressures in patients
with hypertrophic cardiomyopathy. Circulation. 1999;99(2):
25461.
81. Jensen JL, Williams FE, Beilby BJ, et al. Feasibility of
obtaining pulmonary venous flow velocity in cardiac
patients using transthoracic pulsed wave Doppler
technique. J Am Soc Echocardiogr. 1997;10(1):606.
82. Appleton CP. Hemodynamic determinants of Doppler
pulmonary venous flow velocity components: new insights
from studies in lightly sedated normal dogs. J Am Coll
Cardiol. 1997;30(6):156274.
83. Smiseth OA, Thompson CR, Lohavanichbutr K, et al.
The pulmonary venous systolic flow pulseits origin and
relationship to left atrial pressure. J Am Coll Cardiol. 1999;
34(3):8029.
Chapter 59: The Use of Echocardiography to Assess Cardiac Hemodynamics and Guide Therapy
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SECTION 5
Ischemic Heart Disease,
Cardiomyopathies, Pericardial
Disorders, Tumors and Masses
Chapters
Chapter 60
Chapter 61
Chapter 62
Chapter 63
Chapter 64
CHAPTER 60
Echocardiography in Ischemic
Heart Disease
Chetan Shenoy, Hamid Reza Salehi, Francesco F Faletra, Natesa G Pandian
Snapshot
Detecon of Ischemia
Heart Disease
Future
Cardiomyopathy
INTRODUCTION
Coronary artery disease (CAD) is the leading cause of death
for both men and women in the United States. Each year,
an estimated 785,000 Americans will have a new coronary
event, and nearly 470,000 will have a recurrent attack.1 It is
estimated that an additional 195,000 silent first myocardial
infarctions (MIs) occur each year.1 Approximately every
25 seconds, an American will have a coronary event, and
approximately every minute, someone will die of one.1
Noninvasive diagnosis and evaluation of the effects of
CAD are important in risk stratification and guides disease
management. Since the 1980s, echocardiography has been
the mainstay of cardiac imaging in the field of noninvasive
evaluation of CAD.2
Echocardiography has a multifaceted role in ischemic
heart disease.2 It can be used for the noninvasive detection
of chronic ischemia, acute coronary syndrome (ACS),
complications of ACS, and consequences of ischemic heart
disease. The role of echocardiography in ischemic heart
disease is well established, time-tested, and supported
by extensive literature. Advances in echocardiographic
DETECTION OF ISCHEMIA
Stress echocardiography is commonly used for the
detection of chronic ischemia in patients with known or
suspected ischemic heart disease.36 Stress echocardiography can be performed either as an exercise test or as a
pharmacological stress test. For patients who are capable
of performing an exercise test, exercise stress rather than
pharmacological stress is recommended, as the exercise
capacity is a reliable predictor of outcomes.
While either treadmill or bicycle exercise may be
used for exercise stress, the treadmill is widely used in the
United States.37 Symptom-limited exercise according to a
standardized protocol in which the workload is gradually
1290
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Fig. 60.2: Example of a dobutamine exercise stress echocardiogram, demonstrating inferior wall ischemia (arrows). Upper
panelsrest, lower panelsstress. Left panelsdiastolic frames,
right panelssystolic frame.
1291
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 60.3A and B: Example of a wall motion abnormality in the mid-distal anteroseptum in a parasternal three-chamber view (arrows).
(A) Diastolic frame; (B) Systolic frame.
1293
Figs 60.4A and B: Example of a wall motion abnormality in the mid inferoseptum and inferior walls in a short-axis view (arrows).
(A) Diastolic frame, (B) Systolic frame.
Figs 60.5A and B: Example of a wall motion abnormality in the mid-distal lateral wall in an apical four-chamber view (arrows).
(A) Diastolic frame, (B) Systolic frame.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
MECHANICAL COMPLICATIONS OF
MYOCARDIAL INFARCTION
Echocardiography is the primary diagnostic tool for
detection of potentially life-threatening complications
of acute MI.17,18 Portability, immediate availability,
quick imaging, and the detailed information it provides
both on cardiac function and blood flow are critical in
the management of patients with known or suspected
mechanical complications of MI. By helping in diagnosis
and decision-making in these often critically ill patients,
echocardiography can be life-saving.17,18
After the diagnosis of a mechanical complication has
been made, echocardiography could also be used to guide the
intraoperative management (usually with transesophageal
echocardiography) and in assessing the outcome of
complicated surgeries and therapeutic procedures.17,18
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 60.9A and B: Example of a postmyocardial infarction ventricular septal rupture with flow across the defect (arrows).
Figs 60.10A and B: Example of a postmyocardial infarction ventricular septal rupture (asterisk); 2D image (A) and 3D image (B).
1297
Figs 60.11A and B: Examples of papillary muscle rupture (arrows) in two different patients.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
ROLE OF ECHOCARDIOGRAPHY IN
CHRONIC ISCHEMIC CARDIOMYOPATHY
Chronic long-standing CAD, with or without infarction, may
result in progressive deterioration of LV function and other
accompanying pathology such as ischemic MR, increased left
atrial pressure, and so on. Echocardiography is very valuable
in the evaluation of chronic ischemic cardiomyopathy and
associated conditions30 (Figs 60.13A and B).
The assessment of LV systolic function and particularly
regional wall motion is primarily used for the diagnosis of
ischemic cardiomyopathy, determination of the severity,
and prognostication of patients with CAD.30
Ischemic cardiomyopathy is diagnosed on the basis
of LV systolic dysfunction, typically with regional wallmotion abnormalities. There are several possible reasons
for LV systolic dysfunction in patients with chronic
ischemic heart disease.
Figs 60.12A and B: Example of central mitral regurgitation due to ischemic cardiomyopathy [2D image (A) and color Doppler
image (B)].
1299
Figs 60.13A and B: Examples of a left ventricular apical thrombus (arrows) in two different patients, one without (A) and one
with contrast (B).
1300
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
1301
Figs 60.14A and B: Example of Simpsons method of quantification of LV ejection fraction. Diastolic frame (A) and systolic frame (B).
(LV: Left ventricle).
NOVEL ECHOCARDIOGRAPHY
TECHNIQUES IN ISCHEMIC HEART
DISEASE
Three-Dimensional Echocardiography
While 3D echocardiography (3DE) has potential roles
in assessment of ischemia by stress testing, wallmotion abnormalities, left ventricular aneurysm, and
pseudoaneurysm, other complications of MI, the primary
application in patients with ischemic heart disease, is in
the quantification of cardiac chamber volumes, function,
and LV mass.4042
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Speckle-Tracking Echocardiography
Strain imaging potentially has incremental value in the
ability of echocardiography to detect and objectively
qualify ischemia and infarction.44,45 Speckle-tracking
echocardiography as a new noninvasive imaging technique can be helpful for an objective and quantitative
evaluation of global and regional myocardial function.
In contrast to tissue Doppler imaging, it works almost
independently from the angle of insonation and from
cardiac translational movements. This technique is
potentially a better tool to detect myocardial ischemic
segments and also differentiate active contraction from
passive motion such as passive expansion, and recoil and
tethering from adjacent segments.4446
Myocardial Ischemia
The subendocardium is the most vulnerable area to the
effects of hypoperfusion and ischemia; so, LV longitudinal
mechanics (mainly generated by subendocardium) as the
most sensitive parameter may be attenuated in patients
with CAD even at rest. Circumferential strain and twist may
remain normal or show exaggerated compensation for
preserving LV systolic performance. A lower longitudinal
strain value in asymptomatic patients without wallmotion abnormalities can be a strong predictor of stable
ischemic cardiomyopathy. Recently, postsystolic index
has been proposed as an important quantitative marker
for analysis of the ischemic myocardium. LV endocardial
area change ratio by 3D speckle tracking coupled with both
longitudinal and circumferential strain have proposed
to assess induced acute myocardial ischemia in a sheep
model.46
Myocardial Infarction
Longitudinal strains are significantly reduced in patients
with MI, proportionately within the area of infarction,
and correlate closely with peak infarct mass and EF. While
longitudinal strain has been used to detect MI, radial and
circumferential strain values have been used to distinguish
nontransmural infarction from transmural infarction47
(Figs 60.15 and 60.16). The pattern of postsystolic
shortening peak strain after aortic valve closure has been
also reported helpful. In some studies, longitudinal strain
was related to peak levels of cardiac troponin T and the
LV infarct size. It has also been shown that longitudinal
strain correlates with the global and regional extent
(transmurality) of scar tissue as evaluated by contrastenhanced MRI.47
Reperfusion
Longitudinal strain (measured immediately after reperfusion therapy) has been suggested as an excellent
predictor of LV remodeling and adverse events, such as
congestive heart failure and death.45 When combined
with wall-motion scoring, strain rate imaging offered
incremental value over wall-motion scoring alone for
prediction of functional recovery after revascularization.48
Myocardial Viability
Strain imaging as an adjunct to low-dose dobutamine
stress echocardiography has been suggested to assess
1303
Figs 60.15A and B: Polar map derived from speckle-tracking echocardiography showing longitudinal strain in different segments of
two cases, (A) Normal subject (homogeneous yellow) and (B) A patient with a LAD lesion with lower (orange) and positive strain (blue)
in the anteroseptal and septal segments.
Figs 60.16A and B: Speckle-tracking echocardiography demonstrating segmental longitudinal strain in a normal subject (A) and in a
patient with hypokinesis and dyskinesis due to a LAD lesion (B).
FUTURE
Despite the emergence of competing imaging modalities,
echocardiography remains and will continue to be the
primary imaging modality for patients with ischemic
heart disease due to its portability, ease of use,
safety, and technological advancements in terms of
techniques and instrumentation such as tissue Doppler
imaging, 3DE, speckle-tracking imaging, and contrast
echocardiography. Future advances in technology
and increased clinical data will lead to overcoming of
current limitations and further increase the value of
echocardiography in the everyday clinical use for patients
with ischemic heart disease.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
REFERENCES
1. Roger VL, Go AS, Lloyd-Jones DM, et al. Heart disease and
stroke statistics2012 update: a report from the American
Heart Association. Circulation. 2012;125(1):e2220.
2. Gandy WH Jr, Nanda NC. Echocardiography in coronary
artery disease. Curr Opin Cardiol. 1992;7(4):5826.
3. Feigenbaum H. Stress echocardiography: an overview.
Herz. 1991;16(5):34754.
4. Feigenbaum H. The evolution of stress echocardiography.
Cardiol Clin. 1999;17(3):4436, vii.
5. Senior R, Monaghan M, Becher H, et al. British Society
of echocardiography. Stress echocardiography for the
diagnosis and risk stratification of patients with suspected
or known coronary artery disease: a critical appraisal.
Supported by the British Society of Echocardiography.
Heart. 2005;91(4):42736.
6. Pellikka PA, Nagueh SF, Elhendy AA, et al. American
Society of Echocardiography. American Society of
Echocardiography recommendations for performance,
interpretation, and application of stress echocardiography.
J Am Soc Echocardiogr. 2007;20(9):102141.
7. Sicari R, Nihoyannopoulos P, Evangelista A, et al. European
Association of Echocardiography. Stress echocardiography
expert consensus statement: European Association of
Echocardiography (EAE) (a registered branch of the ESC).
Eur J Echocardiogr. 2008;9(4):41537.
8. Ryan T, Williams R, Sawada SG. Dobutamine stress
echocardiography. Am J Card Imaging. 1991;5(2):12232.
9. Porter TR, Xie F. Myocardial perfusion imaging with contrast
ultrasound. JACC Cardiovasc Imaging. 2010;3(2):17687.
10. Chelliah RK, Senior R. Contrast echocardiography: an
update. Curr Cardiol Rep. 2009;11(3):21624.
11. Greaves SC. Role of echocardiography in acute coronary
syndromes. Heart. 2002;88(4):41925.
12. Autore C, Agati L, Piccininno M, et al. Role of echocardiography in acute chest pain syndrome. Am J Cardiol.
2000;86(4A):41G42G.
13. Fuster V, Stein B, Ambrose JA, et al. Atherosclerotic plaque
rupture and thrombosis. Evolving concepts. Circulation.
1990;82(3 Suppl):II4759.
14. Ward RP, Lang RM. Myocardial contrast echocardiography
in acute coronary syndromes. Curr Opin Cardiol. 2002;
17(5):45563.
15. Sia YT, OMeara E, Ducharme A. Role of echocardiography
in acute myocardial infarction. Curr Heart Fail Rep.
2008;5(4):18996.
16. Lewis WR. Echocardiography in the evaluation of patients
in chest pain units. Cardiol Clin. 2005;23(4):5319, vii.
17. Reeder GS. Identification and treatment of complications
of myocardial infarction. Mayo Clin Proc. 1995;70(9):8804.
18. Buda AJ. The role of echocardiography in the evaluation of
mechanical complications of acute myocardial infarction.
Circulation. 1991;84(3 Suppl):I10921.
19. Figueras J, Cortadellas J, Soler-Soler J. Left ventricular
free wall rupture: clinical presentation and management.
Heart. 2000;83(5):499504.
37. McLean DS, Anadiotis AV, Lerakis S. Role of echocardiography in the assessment of myocardial viability.
Am J Med Sci. 2009;337(5):34954.
38. Lang RM, Bierig M, Devereux RB, et al. Chamber
Quantification Writing Group; American Society
of Echocardiographys Guidelines and Standards
Committee; European Association of Echocardiography.
Recommendations for chamber quantification: a report
from the American Society of Echocardiographys Guidelines and Standards Committee and the Chamber
Quantification Writing Group, developed in conjunction
with the European Association of Echocardiography, a
branch of the European Society of Cardiology. J Am Soc
Echocardiogr. 2005;18(12):144063.
39. Nagueh SF, Appleton CP, Gillebert TC, et al. Recommendations for the evaluation of left ventricular diastolic
function by echocardiography. J Am Soc Echocardiogr.
2009;22(2):10733.
40. Pandian NG, Roelandt J, Nanda NC, et al. Dynamic threedimensional echocardiography: methods and clinical
potential. Echocardiography. 1994;11(3):23759.
41. Nanda NC, Miller AP. Real time three-dimensional
echocardiography: specific indications and incremental
value over traditional echocardiography. J Cardiol. 2006;
48(6):291303.
1305
42. Hage FG, Nanda NC. Real-time three-dimensional echocardiography: a current view of what echocardiography
can provide? Indian Heart J. 2009;61(2):14655.
43. Burri MV, Gupta D, Kerber RE, et al. Review of novel
clinical applications of advanced, real-time, 3-dimensional
echocardiography. Transl Res. 2012;159(3):14964.
44. Urbano-Moral JA, Patel AR, Maron MS, et al. Threedimensional speckle-tracking echocardiography: methodological aspects and clinical potential. Echocardiography.
2012;29(8):9971010.
45. Mondillo S, Galderisi M, Mele D, et al. Speckle-tracking
echocardiography: a new technique for assessing myocardial function. J Ultrasound Med. 2011;30(1):7183.
46. Gorcsan J, Tanaka H. Are new myocardial tracking systems
of three-dimensional strain a reality in daily clinical
practice?. Rev Esp Cardiol. 2011;64(12):10829.
47. Geyer H, Caracciolo G, Abe H, et al. Assessment of
myocardial mechanics using speckle tracking echocardiography: fundamentals and clinical applications.
J Am Soc Echocardiogr. 2010;23(4):35169; quiz 453.
48. Gorcsan J 3rd, Tanaka H. Echocardiographic assessment of
myocardial strain. J Am Coll Cardiol. 2011;58(14):140113.
49. Gorcsan J 3rd. Echocardiographic strain imaging for
myocardial viability: an improvement over visual assessment? Circulation. 2005;112(25):382022.
CHAPTER 61
Stress Echocardiography
Azhar Supariwala, Siu-Sun Yao, Farooq A Chaudhry
Snapshot
INTRODUCTION
Stress echocardiography was first introduced by Wann
and coworkers with supine bicycle exercise,1 but the
application was limited until the development of digital
acquisition.2,3 Stress echocardiography is now routinely
used in the diagnosis, risk stratification, and prognosis of
patients with known or suspected coronary artery disease
(CAD).4,5 In recent years, there have been further advances
in the application of stress echocardiography, including
the evaluation of valvular and anatomic abnormalities
of the heart, hemodynamics, myocardial perfusion,
quantitation of wall motion abnormality with speckle
tracking, and three-dimensional (3D) imaging.
FUNDAMENTALS OF STRESS
ECHOCARDIOGRAPHY
The most common application of stress echocardiography
is the detection of flow limiting stenosis. The use of
stress echocardiography to diagnose flow-limiting CAD
is based on a sequence of events known as the ischemic
1307
TYPES OF STRESS
ECHOCARDIOGRAPHY
There are various ways to induce stress such as dynamic
exercise (treadmill or bicycle), pharmacologic stress
(dobutamine, dipyridamole, and adenosine), and pacing
stress.9 Hand grip and leg raise are commonly employed
as an adjunct to the above-mentioned stress modes to
further augment heart rate.10
Exercise Echocardiography
Treadmill exercise using the Bruce protocol is the most
widely used stress modality in the United States. Supine
or semi-supine bicycle stress is widely used in Europe
and in some centers in the United States.11 The images in
treadmill exercise are acquired immediately postexercise
(Figs 61.2A and B). All images are acquired within 3060
seconds, and the entire sequence is repeated to acquire a
second set of images.
Reasons for termination of stress test are symptoms of
typical angina, marked dyspnea, significant ST segment
depression or elevation, sustained arrhythmias, or hemo
dynamic compromise (hypotension or systolic blood
pressure > 220 mm Hg). The advantage of exercise stress
1308
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 61.2A and B: The equipment and set-up for performing a treadmill exercise stress echocardiography.
Contrast Echocardiography
As many as 2030% of patients referred for stress
imaging have suboptimal visualization of the left
ventricular endocardial border echocardiographically.15
Echocardiography contrast agents significantly improve
the bloodendocardial border visualization at rest and
stress.16 Contrast-enhanced echocardiography improves
images in patients with poor acoustic windows and thus
improves inter- and intraobserver variability and diagn
ostic accuracy.15 (Fig. 61.3 and Movie clip 61.1)
INTERPRETATION OF STRESS
ECHOCARDIOGRAPHY
Among expert readers, interpretation of stress echocar
diography has been highly reproducible.20 Interobserver
variability is mostly due to suboptimal image quality and
subtle degrees of wall motion abnormality.21 The resting
echocardiogram may reveal important diagnosis such as
aortic dissection, critical aortic stenosis, and obstructive
hypertrophic cardiomyopathy (HCM) (Fig. 61.4 and Movie
clip 61.2).
A normal response during either exercise or pharma
cological stress echocardiogram is an increase in wall
thickening and endocardial excursion with decrease in left
1309
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Fig. 61.6: The American Society of Echocardiography (ASE) has divided the left ventricle into 17 segments. The schematic
demonstrates the relationship between the coronary artery distribution and the corresponding ASE 17 left ventricular segments.
Analyses of stress echocardiogram should provide the quantitative result according to this model. The four standard echocardiography
views provide evaluation of territories of each of the three main coronary arteries. (Ao: Aorta valve; LA: Left atrium; LV: Left
ventricle; RA: Right atrium; RV: Right ventricle). Source: Reproduced with permission from Vidhun R Echocardiography pocketcard
set, second edition. Brm Bruckmeier Publishing; 2010.
Fig. 61.7: An example of a normal treadmill exercise echocardiogram demonstrating a hyperdynamic response to stress. The
standard format to display exercise stress echocardiography
images. It demonstrates side-by-side rest-stress images. The
heart rate and time of image acquisition postexercise are displayed
for each image. The resting study is on the left and postexercise
study is on the right. The patient had 1-mm ST depressions, which
are likely false-positive ECG changes (Movie clip 61.3). (ECG:
Electrocardiogram; LV: Left ventricle; RV: Right ventricle).
1311
Fig. 61.8: This treadmill exercise echocardiogram demonstrates ischemia. The resting study is normal while the
postexercise images reveal anteroapical wall motion abnormalities. The distribution is consistent with obstructive
coronary artery disease involving left anterior descending artery. (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle) (Movie clip 61.4).
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Table 61.1: Studies with Diagnostic Accuracy of Treadmill, Dobutamine, and Vasodilator Stress Echocardiography Compared to
Coronary Angiographya
Studies
Study Size
116 patients
Sensitivity (%)
NPV(%)
Specificity (%)
PPV (%)
Accuracy (%)
84
63
77
75
80
77
60
78
66
68
61
78
83
72
Two-dimensional
80
82
82
64
77
89
94
40
83
81
74
85
76
88
82
81
Dipyridamole
85
89
87
Dobutamine
86
86
84
Dipyridamole
72
82
77
Treadmill
79
92
80
Meta-analyses:
Gargiulo et al. 201161
Geleijnse et al.
200962
de Albuquerque
Fonseca et al. 200163
1313
Table 61.2: Comparative Accuracy of Stress Echocardiography Versus Nuclear SPECT Imaging
Echocardiography
Studies
Study size
Sensitivity (%)
Specificity (%)
SPECT
Sensitivity (%) Specificity (%)
Meta-analyses:
Heijenbrok-Kal et al.28
351 patients
79
87
88
73
70
90
88
67
Fleischmann et al.30
85
77
87
64
OKeefe et al.65
81
89
90
72
Pharmacologic SE 14 studies
(1,049 patients);
Pharmacologic SPECT 14 studies
81
83
87a
75a
89b
83b
Table 61.3: Factors that Affect Accuracy of Stress Echocardiography for Detecting Hemodynamic Obstructive Coronary Artery
Disease Compared with Coronary Angiography
False Positive
False Negative
Cardiomyopathies
Fig. 61.9: Cardiac event rate per year as a function of wall motion
score index. Worse cardiac event rate is observed with higher
peak wall motion score index.
Source: Reprinted with permission of Elsevier from Yao S, Qureshi
E, Sherrid MV, Chaudhry FA. Practical applications in stress
echocardiography: risk stratification and prognosis in patients
with known or suspected ischemic heart disease. Journal of the
American College of Cardiology. 2003;42(6):1084-90.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Very Low Risk < 0.5 to 1%/year Low Risk < 2%/year Intermediate Risk
2% to 4%/year
Incremental Risk/Other
Independent Predictors
Age
Male gender
Prior history of CHF or MI
Submaximal stress
test (< 85% MPHR)
Single vessel
disease
(LCx or RCA)
Resting ejection
fraction < 45%
Off anti-ischemic
therapy
Extensive ischemia
(45 segments)
On anti-ischemic
therapy
(CAD: Coronary artery disease; CHF: Congestive heart failure; ECG: Electrocardiogram; LCx: Left circumflex; METs: Metabolic equivalent of tasks; MI: Myocardial infarction; MPHR: Maximal predicted heart rate; pWMSI: Peak wall motion score index; RCA: Right
coronary artery).
Source: Modified with permission of Elsevier from Pellikka PA, Nagueh SF, Elhendy AA, et al. Echocardiography recommendations
for performance, interpretation and application of stress echocardiography. Journal of American Society of Echocardiography.
2007;20(9):1021-41.
Fig. 61.11: Schematic for the risk stratification of patients undergoing stress echocardiography. (CAD: Coronary artery disease; CD:
Cardiac death; CRT: Cardiac re-synchronization therapy; EF: Ejection fraction; WMSI: Wall motion score index).
Source: Reprinted with permission of Elsevier from Bangalore S, Yao S, Chaudhry FA. Prediction of myocardial infarction versus cardiac
death by stress echocardiography. Journal of the American Society of Echocardiography. 2009;22(3):261-7).
1315
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Studies
No. of Patients
Abnormal
0.6%/year MI/CD
11.8%/year MI/CD
Bangalore et al.
1.1%/year MI/CD
4.9%/year MI/CD
Wake et al.68
890 patients
(contrast-enhanced DSE)
30 17 months
80% 2-year
(women)
67
Metz et al.69
Meta-analyses
3,021 patients
33 months
0.54%/year-MI/CD
Chaowalit et al.70
96% 5-year
84% 5-year
Biagini et al.
5.9%/year (men)
4.6%/year
(women)
71
72
Sozzi et al.73
7 3.4 years
5 1.7 years
Yao et al.31
1,500 patients
0.9%/year
4.2%/year overall;
1.4%/year TME,
4.7% DSE
Hoque et al.74
Moderate to large
ischemia: 5.5%/
year CD
Sicari et al.75
2.6 3 years
0.9%/year or
92% at ~16 years
Elhendy et al.76
Median 3 years
89.7% at 5 years
CD
Marwick et al.77,78
1.2%/year
1.0%/year
24%/year CD
Krivokapich et al.79
12 months
Poldermans et al.80
Median 3 years
(range 696
months)
1.3%/year
McCully et al.81
Median 2 years
(range 565
months)
0.9%/year
(ACM: All-cause mortality; CD: Cardiac death; Dipy: Dipyridamole; DSE: Dobutamine stress echocardiography; EXSE: Exercise stress
echocardiography; HIV: Human immunodeficiency virus; LVH: Left ventricular hypertrophy; MI: Myocardial infarction; TME: Treadmill exercise stress echocardiography).
1317
Cost-Effectiveness of Stress
Echocardiography in Postmyocardial
Infarction Patients
Cost-effectiveness of the four testing strategies in patients
with prior myocardial infarction was previously evaluated.44
A primary cardiac catheterization strategy (Strategy 1)
was found to be 23% more expensive, a primary stress
electrocardiogram (ECG)/exercise treadmill test strategy
(Strategy 2 and Strategy 3) was 82% more expensive
when compared to a primary stress echocardiography
strategy (Strategy 4). In patients undergoing stress
echocardiography followed by cardiac catheterization
(Strategy 4), the total cost savings was $57,293/patient
compared to a primary cardiac catheterization strategy,
which translated into cost savings of $217/patient correctly
identified. Given the high-risk nature of postmyocardial
infarction patients, it is no surprise that a primary stress
ECG/exercise treadmill test strategy was not cost-effective.
The cost-effectiveness analysis of the postmyocardial
infarction patient study showed that a strategy based on
initial stress testing and cardiac catheterization only in
patients with abnormal stress echocardiogram is ~$217/
patient correctly identified, more economical than a
primary invasive strategy. With a projected incidence of
1.1 million new, recurrent, or silent myocardial infarctions
for 2013 by American Heart Association (AHA),45 the above
data could translate into cost savings of billions of dollars
each year. This is in concordance with previous studies by
1318
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Doppler Hemodynamic
with Stress Echocardiography
Hypertrophic Cardiomyopathy
STRESS ECHOCARDIOGRAPHY:
FUTURE DIRECTIONS
2D Strain, Myocardial Perfusion
and Three Dimensional (3D) Stress
Echocardiography
The interaction of ultrasound energy with the myocardium
results in unique random acoustic speckle patterns.55
Information regarding the motion and displacement
of acoustic speckle of the myocardium can be tracked
automatically throughout the cardiac cycle using sophis
ticated algorithms. The patterns can be used to obtain
strain and strain rate and thus quantification of wall
motion abnormality.55
1319
REFERENCES
1. Wann LS, Faris JV, Childress RH, et al. Exercise crosssectional echocardiography in ischemic heart disease.
Circulation. 1979;60(6):13008.
2. Armstrong WF, Zoghbi WA. Stress echocardiography:
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3. Pellikka PA, Nagueh SF, Elhendy AA, et al. American
Society of Echocardiography. American Society of
Echocardiography recommendations for performance,
interpretation, and application of stress echocardiography.
J Am Soc Echocardiogr. 2007;20(9):102141.
4. Douglas PS, Garcia MJ, Haines DE, et al. ACCF/ASE/
AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011
Appropriate Use Criteria for Echocardiography. A Report
of the American College of Cardiology Foundation
Appropriate Use Criteria Task Force, American Society
of Echocardiography, American Heart Association,
American Society of Nuclear Cardiology, Heart Failure
Society of America, Heart Rhythm Society, Society for
Cardiovascular Angiography and Interventions, Society
of Critical Care Medicine, Society of Cardiovascular
Computed Tomography, and Society for Cardiovascular
Magnetic Resonance Endorsed by the American College
of Chest Physicians. J Am Coll Cardiol. 57(9):112666.
5. Vahanian A, Alfieri O, Andreotti F, et al. Guidelines on the
management of valvular heart disease (version 2012). Eur
Heart J. 33(19):245196.
6. Nesto RW, Kowalchuk GJ. The ischemic cascade: temporal
sequence of hemodynamic, electrocardiographic and
symptomatic expressions of ischemia. Am J Cardiol. 1987;
59(7):23C30C.
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34. Yao SS, Qureshi E, Syed A, et al. Novel stress
echocardiographic model incorporating the extent and
severity of wall motion abnormality for risk stratification
and prognosis. Am J Cardiol. 2004;94(6):7159.
35. Pitt B, Waters D, Brown WV, et al. Aggressive lipid-lowering
therapy compared with angioplasty in stable coronary
artery disease. Atorvastatin versus Revascularization Treat
ment Investigators. N Engl J Med. 1999;341(2):706.
36. Bangalore S, Yao SS, Chaudhry FA. Stress function
index, a novel index for risk stratification and prognosis
using stress echocardiography. J Am Soc Echocardiogr.
2005;18(12):133542.
37. Ghio S, Gavazzi A, Campana C, et al. Independent and
additive prognostic value of right ventricular systolic
function and pulmonary artery pressure in patients with
chronic heart failure. J Am Coll Cardiol. 2001;37(1):1838.
38. Bangalore S, Yao SS, Chaudhry FA. Role of right ventricular
wall motion abnormalities in risk stratification and
prognosis of patients referred for stress echocardiography.
J Am Coll Cardiol. 2007;50(20):19819.
39. Benjamin EJ, DAgostino RB, Belanger AJ, et al. Left atrial
size and the risk of stroke and death. The Framingham
Heart Study. Circulation. 1995;92(4):83541.
40. Bangalore S, Yao SS, Chaudhry FA. Role of left atrial size in
risk stratification and prognosis of patients undergoing stress
echocardiography. J Am Coll Cardiol. 2007;50(13):125462.
41. Bangalore S, Gopinath D, Yao SS, et al. Risk stratification
using stress echocardiography: incremental prognostic
value over historic, clinical, and stress electrocardiographic
variables across a wide spectrum of Bayesian pretest
probabilities for coronary artery disease. J Am Soc
Echocardiogr. 2007;20(3):24452.
42. Yao SS, Bangalore S, Chaudhry FA. Prognostic implications
of stress echocardiography and impact on patient outcomes:
an effective gatekeeper for coronary angiography and reva
scularization. J Am Soc Echocardiogr. 2010;23(8):8329.
43. Bateman TM, OKeefe JH Jr, Dong VM, et al. Coronary
angiographic rates after stress single-photon emission
computed tomographic scintigraphy. J Nucl Cardiol. 1995;
2(3):21723.
44. Bangalore S, Yao SS, Puthumana J, et al. Increm
ental
prognostic value of stress echocardiography over clinical
and stress electrocardiographic variables in patients with
prior myocardial infarction: warranty time of a normal
stress echocardiogram. Echocardiography. 2006;23(6):
45564.
45. Go AS, Mozaffarian D, Roger VL, et al; American Heart
Association Statistics Committee and Stroke Statistics
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Circulation. 2013;127(1):e6e245.
46. Shaw LJ, Marwick TH, Berman DS, et al. Incremental
cost-effectiveness of exercise echocardiography vs. SPECT
imaging for the evaluation of stable chest pain. Eur Heart
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47. Picano E, Pibarot P, Lancellotti P, et al. The emerging role
of exercise testing and stress echocardiography in valvular
heart disease. J Am Coll Cardiol. 2009;54(24):225160.
1321
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59. Jang JY, Sohn IS, Kim JN, et al. Treadmill exercise stress
echocardiography in patients with no history of coronary
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population. Korean Circ J. 2011;41(9):52834.
60. Aggeli C, Felekos I, Roussakis G, et al. Value of real time
three-dimensional adenosine stress contrast echocar
diography in patients with known or suspected coronary
artery disease. Eur J Echocardiogr. 2011;12(9):64855.
61. Gargiulo P, Petretta M, Bruzzese D, et al. Myocardial
perfusion scintigraphy and echocardiography for detecting
coronary artery disease in hypertensive patients: a metaanalysis. Eur J Nucl Med Mol Imaging. 2011;38(11):20409.
62. Geleijnse ML, Krenning BJ, van Dalen BM, et al.
Factors affecting sensitivity and specificity of diagnostic
testing: dobutamine stress echocardiography. J Am Soc
Echocardiogr. 2009;22(11):1199208.
63. de Albuquerque Fonseca L, Picano E. Comparison of
dipyridamole and exercise stress echocardiography for
detection of coronary artery disease (a meta-analysis). Am
J Cardiol. 2001;87(10):11936; A4.
64. Imran MB, Plinks A, Picano E. Head-to-head comparison
of dipyridamole echocardiography and stress perfusion
scintigraphy for the detection of coronary artery disease:
a meta-analysis. Comparison between stress echo and
scintigraphy. Int J Cardiovasc Imaging. 2003;19(1):238.
65. OKeefe JH Jr, Barnhart CS, Bateman TM. Comparison of
stress echocardiography and stress myocardial perfusion
scintigraphy for diagnosing coronary artery disease and
assessing its severity. Am J Cardiol. 1995;75(11):25D34D.
66. Wever Pinzon O, Silva Enciso J, Romero J, et al. Risk
stratification and prognosis of human immunodeficiency
virus-infected patients with known or suspected coronary
artery disease referred for stress echocardiography. Circ
Cardiovasc Imaging. 2011;4(4):36370.
67. Bangalore S, Yao SS, Chaudhry FA. Usefulness of stress
echocardiography for risk stratification and prognosis of
patients with left ventricular hypertrophy. Am J Cardiol.
2007;100(3):53643.
68. Wake R, Takeuchi M, Yoshikawa J, Yoshiyama M. Effects
of gender on prognosis of patients with known or susp
ected coronary artery disease undergoing contrastenhanced dobutamine stress echocardiography. Circ J.
2007;71(7):106066.
69. Metz LD, Beattie M, Hom R, et al. The prognostic value
of normal exercise myocardial perfusion imaging and
exercise echocardiography: a meta-analysis. J Am Coll
Cardiol. 2007;49(2):22737.
70. Chaowalit N, McCully RB, Callahan MJ, et al. Outcomes
after normal dobut
amine stress echo
cardiography and
CHAPTER 62
Squatting Stress
Echocardiography
Premindra AN Chandraratna, Dilbahar S Mohar, Peter Sidarous
Snapshot
Squatting Echocardiography
INTRODUCTION
Regional mismatch between coronary oxygen supply
and myocardial demand results in myocardial ischemia.
In such ischemic settings, wall motion abnormalities
(WMAs) detectable by echocardiography manifest early
as diastolic and subsequently systolic changes. Moreover,
functional wall abnormalities are early changes in the
well-described ischemic cascade, which concludes with
later surrogates including electrocardiography (ECG)
changes and subsequently overt chest pain.1 As such,
stress echocardiography (SE) has an established utility for
the detection of significant coronary artery disease (CAD)
with a notable accuracy of 8090%, which is superior to that
of exercise electrocardiographic testing and comparable to
that of nuclear stress imaging.2
Stress echocardiography is performed either with
exercise on a treadmill or bicycle or by infusion of a
pharmacological agent such as dobutamine, adenosine,
dipyridamole, or transes
ophageal atrial pacing. Each
of these techniques has advantages and disadvantages.
Treadmill or bicycle exercise echocardiography permits
assessment of both myocardial ischemia and functional
capacity. However, this technique may have limited
echocardiographic utility in patients in whom peak
exercise is not attainable or in patients with single vessel or
1324
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Fig. 62.1: End-systolic frames during standing (left) and squatting (right) in a normal subject. Note the triangular shape of the
apex during standing and squatting. (LV: Left ventricle). (Movie
clip 62.1).
Source: Reproduced with permission from Ref. 10.
SQUATTING ECHOCARDIOGRAPHY
Squatting echocardiography has emerged as a promising
modality which augments afterload and preload with little
or no change in cardiac contractility. Moreover, as afterload
and preload are major determinants of myocardial oxygen
consumption, patients with significant CAD develop acute
WMAs during squatting, which reversibly resolve upon
arising. Lewis et al. studied the effects of squatting on
hemodynamic parameters and left ventricular (LV) dime
nsions in normal subjects.8 They observed that squatting
produced increased LV cavity dimensions and increase
in mean blood pressure. There was an increase in stroke
index and cardiac index.
Chandraratna and colleagues have extended previous
observations on squatting echocardiography and demon
strated that squatting produces LV WMA in patients with
CAD.
Fig. 62.2: Echocardiogram of a patient with left ventricular (LV) function normal in the standing position. The LV apex had a triangular
appearance at end-systole. An extensive wall motion abnormality
developed during squatting (arrows). The distal posterior septum,
apex, and distal posterolateral wall became akinetic, and the distal
half of the LV became dilated. The wall motion abnormalities and the
acute left ventricular remodeling (AVLRM) normalized on standing.
(Movie clip 62.2).
Source: Reproduced with permission from Ref. 10.
Squatting Echocardiography:
Results/Observations
Squatting Induces LV WMA in Areas
Subtended by Stenotic Coronary Arteries
The study populations consisted of 15 normal male
subjects (Group 1) and 42 males subjects (Group 2) who
had coronary angiography.9 Each patient underwent
squatting echocardiography testing as per protocol, and
standard echocardiographic views were attained and
interpreted by an expert echocardiography reader blinded
to angiographic results.
Group 1 subjects had normal LV global and regional
function while standing. There were no WMA while
squatting (Fig. 62.1 and Movie clip 62.1). In Group 2, five
patients had a baseline WMA. New or worsening WMA
occurred during squatting in 35 patients. Twelve patients
developed a WMA in the left anterior descending coronary
artery territory, five had WMA in the circumflex coronary
artery territory, seven had WMA in the right coronary
artery territory, and seven had normal wall motion. Eleven
had WMA in multiple territories (Fig. 62.2 and Movie
clip 62.2). All WMA resolved on standing within 1 min.
None of these patients developed chest pain, arrhythmias,
or hypotension.
1325
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Table 62.1: Heart Rate and Blood Pressure Response to Squatting versus Dobutamine11
Total Cohorts
Standing
Squatting
p-value
Standing versus
Squatting
Dobutamine
p-value
Squatting versus
Dobutamine
64 10
73 10
<0.0001
123 18
<0.0001
121 11
136 11
<0.0001
159 23
<0.0001
79 6
86 7
<0.001
88 10
<0.001
Source: Reprinted with permission from Chandraratna PA, Kuznetsov VA, Mohar DS, et al. Comparison of squatting stress echocardiography and dobutamine stress echocardiography for the diagnosis of coronary artery disease. Echocardiography. 2012; 29(6):6959.
Advantages
Furthermore, there are numerous advantages to squatting
as a stress test. The absence of marked changes in heart
rate during squatting makes comparison with the baseline
echocardiogram and interpretation of stress-induced
WMA easier than when tachycardia is present. Ventricular
dilatation that occurs with squatting facilitates analysis
of wall motion. The rapid recovery of wall motion after
squatting reduces adverse sequelae. No ventricular
arrhythmias, chest pain, or hypotension were noted
during squatting. Additionally, the lack of prominent and
sustained heart rate and blood pressure increases, which
are known characteristics of other stress imaging such as
dobutamine or treadmill testing, further validates squat
echocardiography as a safer modality with fewer adverse
risks.
Limitations
There are some limitations to squatting as a stress test.
Patients with knee or hip disease or extreme obesity may
not be able to squat. The sonographer also has to squat
during the procedure, and only those with adequate
echocardiography windows are suitable for testing.
Squatting SE possesses similar limitations as com
pared to treadmill testing with respect to obesity and
orthopedics. However, unless patients have morbid obesity
or advanced orthopedic restrictions, squat imaging is still
attainable with limited effort as squatting is essentially
a static position. The same cannot be said for treadmill
testing in which certain heart rate and blood pressure end
point parameters need to be achieved for proper treadmill
assessment.
CONCLUSION
In patients with significant CAD, squatting echocar
diography has emerged as a unique stress modality, which
REFERENCES
1. Nesto RW, Kowalchuk GJ. The ischemic cascade: temporal
sequence of hemodynamic, electrocardiographic and
symptomatic expressions of ischemia. Am J Cardiol. 1987;
59(7):23C30C.
2. Marwick TH. Stress echocardiography. Heart. 2003;89(1):
11318.
3. Marwick TH, Nemec JJ, Pashkow FJ, et al. Accuracy and
limitations of exercise echocardiography in a routine
clinical setting. J Am Coll Cardiol. 1992;19(1):7481.
4. Sicari R, Nihoyannopoulos P, Evangelista A, et al; European
Association of Echocardiography. Stress echocardiography
expert consensus statement: European Association of
Echocardiography (EAE) (a registered branch of the ESC).
Eur J Echocardiogr. 2008;9(4):41537.
5. Mertes H, Sawada SG, Ryan T, et al. Symptoms, adverse
effects, and complications associated with dobutamine
stress echocardiography. Experience in 1118 patients. Cir
culation. 1993;88(1):1519.
6. Douglas PS, Garcia MJ, Haines DE, et al. ACCF/ASE/
AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011
Appropriate Use Criteria for Echocardiography. A Report
of the American College of Cardiology Foundation
Appropriate Use Criteria Task Force, American Society of
Echocardiography, American Heart Association, American
Society of Nuclear Cardiology, Heart Failure Society of
America, Heart Rhythm Society, Society for Cardiovascular
Angiography and Interventions, Society of Critical Care
Medicine, Society of Cardiovascular Computed Tomo
graphy, Society for Cardiovascular Magnetic Resonance
American College of Chest Physicians. J Am Soc Echo
cardiogr. 2011;24(3):22967.
1327
CHAPTER 63
Three-Dimensional Stress
Echocardiography
Rajesh Ramineni, Masood Ahmad
Snapshot
Three-Dimensional Transducers
Postacquisition Analysis
Visualization
Echocardiography
Future Directions
INTRODUCTION
Stress testing has been in use for a very long time in the
diagnosis of coronary artery disease (CAD), in testing
functional capacity, and in assessing prognosis. Stressinduced changes in electrocardiogram were initially
used to evaluate ischemia until the introduction of
imaging techniques, which offered higher sensitivity and
specificity. We reported the use of isometric exercise
during M-mode echocardiography in the detection of left
anterior descending (LAD) artery disease as far back as
1976.1 The value of treadmill exercise echocardiography in
the diagnosis of CAD was subsequently demonstrated.2 A
plethora of reports on the use of stress echocardiography
followed.26 Ever since, two-dimensional stress echocardi
ography (2DSE) has become an established technique
in assessing patients with CAD. Since the stress-induced
transient left ventricular (LV) wall motion abnormality
needs to be obtained rapidly,7 the technique of acquiring
TWO-DIMENSIONAL STRESS
ECHOCARDIOGRAPHY
As an established technique in the assessment of
patients with CAD, 2DSE is commonly used in many
THREE-DIMENSIONAL
TRANSDUCERS
Initial attempts to obtain 3D images were based on
reconstruction of multiple 2D images acquired by
manually tilting the transducers at various angles around a
fixed plane (Figs 63.1A and B). However, the acquisition by
1329
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Advantages:
Shorter acquisition time
User-friendly and less operator dependent
Good interobserver agreement. Reproducible
Precise alignment/anatomically correct tomographic section. More accurate comparison of matched rest and stress images
Full-volume acquisition of entire true LV. Multiple sections of the LV and apical region essentially eliminate problems related to
off-axis image acquisition or LV foreshortening
Quantitative. Assessment of LV volume and EF comparable to CMR
Limitations:
Lower spatial and temporal (frame/volume rate) resolution especially at peak stress
Influenced by respiration, patient motion, and significant variation in heart rate causing image artifacts
Suboptimal anterior and lateral wall visualization related to larger transducer footprint
Longer offline data analysis time
(CMR: Cardiac magnetic resonance; EF: Ejection fraction; LV: Left ventricle).
THREE-DIMENSIONAL
IMAGE ACQUISITION
The introduction of matrix array transducers and parallel
processing allowed real time acquisition of 3D images as
opposed to reconstruction from spatially oriented and
sequentially obtained 2D images. Initially, the matrix array
had 256 elements that generated a 60 60 pyramidal
volume. Although the stress images had limited resolution,
the ability to obtain multiple views in either orthogonal
THREE-DIMENSIONAL
STRESS PROTOCOL
The early versions of the 3D equipment lacked combined
2D and 3D imaging. Therefore, in order to obtain 2D and
3D images at baseline and at peak stress, the transducers
had to be switched to obtain both sets of images. With
the newer systems, the transducers have both 2D and
3D imaging capabilities. Both sets of images can also be
obtained by simply switching the acquisition modes.
Alternatively, all 2D images can be derived from the
3D data set. The protocol for imaging 3D data sets with
routinely performed 2D stress imaging remains variable
from laboratory to laboratory. Our approach is to use an
integrated 2D/3D stress protocol that includes 3D images
in the standard 2D stress imaging protocol. Essentially,
3D full-volume acquisitions are obtained at baseline after
capturing standard 2D images from multiple windows. At
this time, we obtain 3D acquisitions from the apical and
parasternal windows. These steps are repeated at peak
stress.
POSTACQUISITION ANALYSIS
A single, full-volume acquisition of RT3D can be
analyzed in various formats. A simultaneous display of
major orthogonal views can be created. This is called
the multiplane view. We are able to obtain parasternal
long- or short-axis or apical four-, two- or three-chamber
views by this technique. Multiple short-axis 2D images
(nine equidistant images in general) can also be obtained
from apex to base. This is called the multislice view.
(Movie clips 63.1 to 63.4). Studies have shown that
specificity of detection of CAD and accuracy of identifying
right coronary lesions were significantly greater with the
use of multislice view.26 This advantage was attributed to
better visualization of basal wall motion abnormalities
and the acquisition of correct short-axis views. This view
is also associated with greater reduction in interobserver
variances.26 Analysis of the LV wall motion can be done by
the 16- or 17-segment model as recommended by the ASE.
The images are analyzed in a complementary fashion;
1331
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Table 63.2: Diagnostic Sensitivity and Specificity of Three-Dimensional (3D) Versus Two-Dimensional (2D) in Detecting Coronary
Artery Disease (CAD)
No.
Validation
58
DSE
56
78
8
24
22
2D Sen.
Sp.
3D Sen.
Sp.
Coronary angiography
79
88
DSE
Thalium201-SPECT
86
83
80
DSE
None
58
75
56
DSE
Coronary angiography
73
78
93
89
71
DSE-3D multiplane
Coronary angiography
72
72
DSE-3D multislice
Coronary angiography
77
95
Treadmill exercise-2D
Coronary angiography
83
65
Treadmill exercise-3D
Coronary angiography
40
84
Treadmill exercise-3D+
CFM
Coronary angiography
55
78
107
Dipyridamole
None
78
91
80
87
30
Adenosine 2D
Tc 99m Sestamibi
SPECT
92
75
Adenosine Live 3D
Tc 99m Sestamibi
SPECT
91
69
Tc 99m Sestamibi
SPECT
90
79
90
32
33
(CFM: Contraction front mapping; DSE: Dobutamine stress echocardiography; No.: Number of subjects; Sen.: Sensitivity;
Sp.: Specificity; SPECT: Single-photon electron-computed tomography; Tc: Technetium).
1333
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
CONTRAST IN THREE-DIMENSIONAL
STRESS TESTING
Given the limited resolution of 3D when compared to 2D,
use of contrast agents to improve the visualization of the
endocardial border makes theoretical sense. Pulerwitz,
in an initial study of 14 patients, noted that ultrasound
contrast significantly increased the proportion of segments
adequately visualized during rest and peak dobutamine
infusion (91%98%, P = 0.001, and 87%99%, P = 0.001,
respectively).16 There was almost complete concordance
between observers (96.9% at rest and 98.2% at peak
stress with almost no interobserver variability), whereas
noncontrast studies had much lower agreement (84.4%
at rest and 79.9% at peak stress with kappa values less
than 0.4).
Nemes et al. in 2007, studied the use of contrast in
36 patients undergoing routine stress test for stable chest
pain.34 The images obtained with and without contrast
were compared for image quality index, and wall motion
was assessed using the standard 17-segment LV model.
It was noted that myocardial segment visualization
improved from 76% to 90% with the use of contrast, and the
image quality index improved from 2.2 to 3.1. Agreement
on coronary territory of ischemia improved from 79%
to 88%. Study agreement on myocardial ischemia also
improved from 72% to 89%. However, this study used the
larger transducer (X4 24 20 mm) with a bigger footprint
resulting in less favorable outcome with the use of
conventional RT3DE.
FUTURE DIRECTIONS
Further developments in transducer technology and
processing techniques should allow 3D acquisitions at
higher volume rates at high heart rates during stress.
Single beat acquisitions of full volumes will avoid
the potential for artifacts. Automated software for
side-by-side display of baseline and stress 3D images will
facilitate interpretation of 3DSE. Parametric imaging may
provide an entirely new approach in mapping ischemic
regions of the left ventricle. These developments should
1335
CONCLUSION
3DSE is an exciting technology that is rapidly evolving and
currently applied as an adjunctive technique to 2DSE.
The availability of 3D images is tremendously useful
in evaluating questionable areas of abnormality seen
in 2DSE and in more accurately assessing the changes
induced by ischemia.36 In the near future, 3DSE may be
applied independent of the traditional 2DSE resulting in a
comprehensive diagnostic stress test that is more efficient,
quantitative, and reproducible in the diagnosis of CAD.
REFERENCES
1. Ahmad M, Watson, LE. Application of stress echocardi
ography in the diagnosis of left anterior descending
coronary artery disease. Clin Res. 1976;515A.
2. Maurer G, Nanda NC. Two dimensional echocardiographic
evaluation of exercise-induced left and right ventricular
asynergy: correlation with thallium scanning. Am J Cardiol.
1981;48(4):7207.
3. Innocenti F, Caldi F, Tassinari I, et al. Prognostic value of
exercise stress test and dobutamine stress echo in patients
with known coronary artery disease. Echocardiography.
2009;26(1):19.
4. Olmos LI, Dakik H, Gordon R, et al. Long-term prognostic
value of exercise echocardiography compared with exercise
201Tl, ECG, and clinical variables in patients evaluated for
coronary artery disease. Circulation. 1998;98(24):26792686.
5. Marwick TH. Stress echocardiography. Heart. 2003;89(1):
1138.
6. Abreo G, Lerakis S, Ahmad M. Use of exercise echocardio
graphy to evaluate patients with chest pain. Am J Med Sci.
1998;316(5):34550.
7. Armstrong WF, Pellikka PA, Ryan T, et al. Stress
echocardiography: recommendations for performance and
interpretation of stress echocardiography. Stress Echocar
diography Task Force of the Nomenclature and Standards
Committee of the American Society of Echocardiography.
J Am Soc Echocardiogr. 1998;11(1):97104.
1336
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
CHAPTER 64
Echocardiographic Assessment of
Coronary ArteriesMorphology and
Coronary Flow Reserve
Karina Wierzbowska-Drabik, Jarosaw D Kasprzak
Snapshot
INTRODUCTION
Coronary arteries are visible during standard echocardiographic examination. However, small size and vigorous
motion of epicardial coronary segments during respiratory
and cardiac cycle pose a significant challenge for all noninvasive imaging methods. Therefore, direct evaluation of
coronary arteries has not become a part of routine transthoracic echocardiographic (TTE) examination despite
significant technical improvements leading to improved
quality of imaging. These limitations are less evident in
transesophageal echocardiogram (TEE). Recent progress
in Doppler sensitivity encouraged routine use of distal
coronary flow velocity and vasodilator-induced velocity
reserve measurements.
VISUALIZATION OF
CORONARY ARTERIES
Transthoracic Echocardiography
Visualization of proximal segments of coronary arteries
is possible in the majority of TTE examinations. Good-
1338
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Fig. 64.1: A typical image of proximal coronary arteries in parasternal short-axis view. (Ao: Aorta, LCA: Left coronary artery;
RCA: Right coronary artery; RV: Right ventricle).
Transesophageal Echocardiography
Higher ultrasound beam frequency (thus higher resolution) and anatomical proximity create opportunities for
improved imaging of proximal coronary arteries (especially
left) from the transesophageal window, including better
anatomical definition of possible lesions. TEE enables
visualization of proximal coronary arteries in more than
90% of studies and offers interpretable recordings of flow
in 85%, 65%, and 58% of LAD, Cx, and RCA , respectively.
As opposed to TTE, TEE allows the assessment of vessel
morphology including the identification of luminal
narrowing.6 Importantly, from the clinical point of
view, LMCA is evaluable in nearly in 100% for twodimensional and 88% for flow imaging by experienced
observer during TEE study.7 The technique of TEE
assessment of coronary ostia is based on the systematic
Chapter 64: Echocardiographic Assessment of Coronary ArteriesMorphology and Coronary Flow Reserve
1339
Fig. 64.4: Normal left main coronary artery with bifurcation visualized in transesophageal echocardiography (TEE). (Ao: Aorta; LA:
Left atrium; RVOT: Right ventricle outflow tract).
Fig. 64.5: Normal proximal right coronary artery visualized in twoand three-dimensional echocardiography including ostial en-face
views and flow recording. (Ao: Aorta; RA: Right atrium; RCA: Right
coronary artery; RVOT: Right ventricle outflow tract; LA: Left atrium).
1340
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 64.7A and B: Examples of stenosis in circumflex (Cx; A) and left anterior descending (LAD; B) detected by transesophageal echocardiography (TEE) color Doppler. (Ao: Aorta; RVOT: Right ventricle outflow tract; LCA: Left coronary artery; Cx: Circumflex artery; LAD:
Left anterior descending; LA: Left atrium).
Fig. 64.8: Stenotic distal left main artery (LMA) with accelerated
flow spectrum recorded by transesophageal echocardiography
(TEE). (Ao: Aorta; RVOT: Right ventricle outflow tract; LA: Left
atrium; LCA: Left coronary artery).
Chapter 64: Echocardiographic Assessment of Coronary ArteriesMorphology and Coronary Flow Reserve
1341
Fig. 64.9: Proximal left coronary artery analyzed in three-dimensional echocardiography including ostial en-face views and multislice
imaging. (Ao: Aorta; AV: Aortic valve; RV: Right ventricle; LA: Left atrium; LCA: Left coronary artery).
Table 64.1: The Optimal Views for Proximal Segments of Coronary Arteries and Proposed Cut-Off Values for the Assessment of
Significant Stenosis
Coronary Artery
TTE-Optimal View
TEE-Optimal View
Proposed Cut-Off
Values11
Proposed Cut-Off
Values12
LMCA
LAD
Cx
RCA
Parasternal long-axis,
parasternal short-axis
(Cx: Circumflex; LAD: Left anterior descending; LMCA: Left main coronary artery; RCA: Right coronary artery; TEE: Transesophageal
echocardiography; TTE: Transthoracic echocardiography).
1342
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Fig. 64.10: Distal left coronary artery (LCA) flow recorded in color
and spectral Doppler, modified apical four-chamber view. (Ant IVS:
Anterior interventricular septum; LCA: Left coronary artery; LV: Left
ventricle).
Table 64.2: The Conditions Decreasing CFR in the Absence of Significant Stenoses in Epicardial Arteries
Hypertrophic cardiomyopathy
Hypertrophy in arterial hypertension
Aortic stenosis
Aortic insufficiency
Dilated cardiomyopathy
Diabetes
Syndrome X
Increased blood viscosity: policythemia, macroglobulinemia
Hypercholesterolemia
For some of these conditions (e.g. aortic valve disease, hypertension, hypercholesterolemia) surgical or medical treatment could
reverse coronary flow reserve (CFR) impairment.10
Chapter 64: Echocardiographic Assessment of Coronary ArteriesMorphology and Coronary Flow Reserve
1343
CONGENITAL ABNORMALITIES OF
THE CORONARY ARTERIES
The prevalence of coronary arteries anomalies ranges
from about 1 to 5.6% and the most frequent anomaly is the
Cx branch originating from the RCA or the right sinus of
Fig. 64.12: Normal coronary velocity flow reserve measured in
3639
distal left anterior descending (LAD)coronary flow velocity Valsalva, found in 0.48% of the invasive angiographies.
The clinical presentation may be silent or they may cause
reserve (CFVR) = 3.0.
angina, arrhythmia, syncope, myocardial infarction, and
40
in subendocardial layer, with higher CFVR values in sudden cardiac death. Some anomalies of coronary
subepicardial than subendocardial regions.27 The value arteries origin including the LMCA arising from the right
of adding CFVR (usually LAD) to wall motion assessment sinus of Valsalva (although less common than the origin of
lies in improved sensitivity without specificity loss.28 RCA from the left sinus of Valsalva (Fig. 64.14), may have
CFVR is recommended as a component of state-of-the- fatal consequences related to slit-like orifice narrowing,
art standard stress echo protocol with dipyridamole and sharp angulation, and risk of intra-arterial course between
is most practical when clinical questions regarding the the aorta and pulmonary artery, with threatened sudden
specific anatomical locations arise. Recently published cardiac death during exercise. The diagnosis of coronary
study has shown high accuracy of coronary flow reserve anomalies by echocardiography has been overshadowed
< 2 assessed by TTE for the detection of significant by magnetic resonance or computed echocardiography;
restenosis after stent implantation, again with cutoff value however, it remains a useful option, is radiation-free,
< 2. 0 for three major coronary arteries.29 The diagnostic and allows real-time assessment of coronary anatomy
value of TTE coronary flow reserve is high and similar to and flow. While TTE remains challenging in some cases,
TEE images usually unveil realistic coronary anatomy.
320-row computed tomography.30
The impairment of coronary flow reserve with cut- Such composite diagnostic approach, including invasive
off value < 1.7 assessed during the 24-hour period after coronary angiography or intracoronary ultrasound as
primary coronary intervention was also documented necessary, may be necessary to define the exact course of
as the predictor of left ventricular remodeling early coronary arteries, elucidate possible pathomechanisms
after anterior myocardial infarction.31 Decreased aiding in therapeutic decision-making. This may be
coronary flow reserve < 2.6 with shortened < 840 ms especially valuable when overlap of congenital and
diastolic deceleration time were also related to higher acquired atherosclerotic coronary disease comes into
incidence of cardiac events in patient after heart play.41 Identification of aberrant left coronary artery with
transplantation, defined as cardiac death, heart failure, interarterial course alone represents an indication for
and stent implantation.32 Reduced long-term survival surgical intervention, whereas in anomalous origin of the
was also described in patients with coronary flow reserve RCA more percutaneous interventions may be considered.
< 2 in dilated cardiomyopathy together with such known Finally, echocardiography may provide the valuable
predictors as increased wall motion score index (WMSI) noninvasive and radiation-free tool for the follow-up and
monitoring of coronary intervention results.42
and mitral regurgitation.33
Coronary fistulas are uncommon coronary pathology.
In the context of intracoronary stem cell therapy, early
injection in the infarct-related coronary bed does not seem Pediatric type features low-resistance connection of the
1344
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Fig. 64.13: Low coronary velocity flow reserve measured in distal left anterior descending (LAD)coronary flow velocity reserve (CFVR)
= 1.7 in a hypertensive diabetic patient free of epicardial coronary disease.
Fig. 64.14: Coronary anomalyright coronary artery (RCA) originating from the left sinus of Valsalva with interarterial course. Mild flow
abnormality (turbulence) visualized in transesophageal echocardiography (TEE). (RCA: Right coronary artery; RVOT: Right ventricle
outflow tract).
SUMMARY
The evaluation of coronary arteries by modern echocardiography enables routine noninvasive detection of
congenital anomalies and significant proximal stenosis,
especially in LMCA, which underscore the role of this
method as noninvasive and valuable screening tool in
these frequently dangerous setting. Functional assessment
of coronary stenosis by noninvasive CFVR contributes to
physiological stratification of luminal coronary narrowing,
and echocardiogram may support monitoring of coronary
artery interventional treatment, offering additional
prognostic information.
Chapter 64: Echocardiographic Assessment of Coronary ArteriesMorphology and Coronary Flow Reserve
1345
Fig. 64.15: Pediatric type coronary fistula from the right coronary
artery to the right atrium. (RV: Right ventricle; RCA: Right coronary
artery; LV: Left ventricle).
Fig. 64.16: Minor left anterior descending (LAD) fistulous connection to the proximal pulmonary trunk (diastolic color flow visible) with
corresponding angiogram. (Ao: Aorta; RVOT: Right ventricle outflow tract; MPA: Major pulmonary artery; LA: Left atrium; LAD: Left
anterior descending; LCA: Left coronary artery).
REFERENCES
1. Anjaneyulu A, Raghu K, Chandramukhi S, et al. Evaluation
of left main coronary artery stenosis by transthoracic echocardiography. J Am Soc Echocardiogr. 2008;21(7):85560.
2. Vegsundvg J, Holte E, Wiseth R, et al. Transthoracic
echocardiography for imaging of the different coronary
1346
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Chapter 64: Echocardiographic Assessment of Coronary ArteriesMorphology and Coronary Flow Reserve
31.
32.
33.
34.
35.
36.
1347
CHAPTER 65
Echocardiography in Hypertrophic
Cardiomyopathy
Dan G Halpern, Mark V Sherrid
Snapshot
Dieren al Diagnosis
INTRODUCTION
Hypertrophic cardiomyopathy (HCM) is a genetic disorder
with clinically unexplained myocardial hypertrophy (most
commonly of the interventricular septum) that occurs in
the absence of a hemodynamic cause. HCM predisposes
to symptoms, dynamic left ventricle obstruction, and
infrequently, to life-threatening arrhythmias.1 It is the most
common inherited disorder among cardiovascular diseases
(1:500) and is the leading cause of sudden cardiac death
(SCD) in young adults.1 Among its previous names are
idiopathic hypertrophic subaortic stenosis and muscular
subaortic stenosis. The preferred nomenclature is HCM,
either obstructive, or nonobstructive. HCM is inherited with
autosomal dominant transmission; currently mutations in
11 genes coding for various cardiac sarcomeric proteins are
associated with HCM.2,3 Varying phenotypic expressions
and marked heterogeneity is a hallmark of HCM (Figs 65.1A
to D). Microscopically, HCM is characterized by myocyte
hypertrophy and myocytic disarray interlaced with fibrosis.
Transthoracic echocardiography (TTE) is the most
powerful tool for the diagnosis, management, and followup of HCM.4 TTE demonstrates the site and extent of
hypertrophy, and delineates and quantifies obstruction.
Before making the diagnosis, it is imperative to rule out
more common secondary causes of concentric hypertrophy
1349
Figs 65.1A to D: Major patterns of hypertrophy in hypertrophic cardiomyopathy (HCM). Schematic (left panel) versus echocardiographic
images (right panel). (A) Anterior septal hypertrophy; (B) Subaortic septal bulge; (C) Apical; (D) Midleft ventricular with obstruction.
Source: Reproduced in part with permission from Shah A et al. Severe symptoms in mid and apical hypertrophic cardiomyopathy.
Echocardiography. 2009;26:92233.
1350
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Fig. 65.3: Systolic anterior motion (SAM) of the mitral valve. SAM
of the mitral valve, drawn from an apical five-chamber view, as it
proceeds in early systole.
Source: Reproduced with permission from Sherrid MV, et al. An
echocardiographic study of the fluid mechanics of obstruction
in hypertrophic cardiomyopathy. J Am Coll Cardiol. 1993:22;
81625.
1351
Figs 65.4A and B: The pushing force of flow. (A) The dark blue, low-velocity flow behind the mitral valve pushes the leaflets
into the septum, before high-velocity flow has occurred in the outflow tract; (B) The spinnaker is pushed by the wind that strikes
its undersurface. Image of Stanley Rosenfeld reproduced with permission, The Rosenfeld Collection, Mystic Seaport Museum.
Source: Reproduced with permission from Sherrid MV, et al. Pathophysiology and treatment of hypertrophic cardiomyopathy. Prog
Cardiovasc Dis. 2006;49:12351. (LA: Left atrium; LV: Left ventricle; MV: Mitral valve; LVOT: Left ventricular outflow tract.
1352
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
1353
1354
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 65.10A to C: Anomalous papillary muscle inserting in the middle of the anterior mitral valve leaflet and causing left ventricular
outflow tract (LVOT) obstruction. Left panels show the obstructing papillary muscle on parasternal (A) and short-axis views (B)
and the anatomical specimen. Center (A,B,C) and right panels (A,B) show inadequate resections in patients with this pathology
(center C and right B). Because the septal resections have not been carried far enough down the septum, and because the papillary
muscle abnormalities have not been addressed, there is still severe residual obstruction shown by the arrowheads in both cases.
Source: Reproduced with permission from Klues HG, et al. Anomalous insertion of papillary muscle directly into anterior mitral leaflet in
hypertrophic cardiomyopathy. Significance in producing left ventricular outflow obstruction. Circulation. 1991;84:118897, and Maron
BJ, et al. Pitfalls in clinical recognition and a novel operative approach for hypertrophic cardiomyopathy with severe outflow obstruction
due to anomalous papillary muscle. Circulation. 1998;98:25058.
1355
Fig. 65.11: Patient with obstructive hypertrophic cardiomyopathy and latent obstruction. There is no left ventricle (LV) outflow gradient at rest. Gradient rises to 49 mm Hg after Valsalva, to 74 mm Hg after standing, and to 144 mm Hg after treadmill
exercise. In 30% of cases, we found a higher standing than Valsalva gradient, as in this patient. Of the 56 patients (57%)
with resting gradients < 30 mm Hg, standing provoked a gradient
30 mm Hg in 23 patients (41%), thus placing them in the
domain of obstructive HCM. Standing gradient is recommended on the index echocardiogram in every patient with HCM or SAM.
Source: Reproduced with permission from Joshi S, et al. Standing and exercise Doppler echocardiography in obstructive hypertrophic
cardiomyopathy: the range of gradients with upright activity. J Am Soc Echocardiogr. 2011;24:7582.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Diastolic Dysfunction
Impaired relaxation is widely present in HCM as well as
impaired compliance from myocardial fibrosis.54,55 Global
and segmental assessment of diastolic function should
be assessed by measurement of the transmitral inflow
velocities, tissue Doppler velocities and pulmonary vein
flow velocities, as well as left atrial size. Tissue Doppler
velocities are decreased in HCM.56 However, the E/e' ratio
has limited application in HCM and does not correlate well
with LV filling pressures.54 The thickened septum usually
has the greatest segmental diastolic dysfunction.57 The
presence of a transmitral A-wave should also be noted. Its
absence could denote atrial fibrillation or atrial stunning,
which might be an important source of symptoms in
patients with restrictive physiology. Furthermore, tissue
Doppler imaging in a gene-positive HCM relative with
normal wall thickness might denote latent subclinical
cardiomyopathy by demonstrating reduced systolic
(S') and early filling (e') velocities.58 Strain imaging may
provide additional information on segmental dysfunction
and latent myopathy.59 In patients with LVOT obstruction,
the relief of the obstruction improves diastolic relaxation.60
Otherwise, no pharmacological therapy has been shown
convincingly to directly improve diastolic function in
HCM.61,62
MIDLEFT VENTRICULAR
HYPERTROPHIC CARDIOMYOPATHY
An uncommon but clinically important form of HCM has
a unique midventricular distribution of hypertrophy,
and in many patients subsequent midcavity obstruction
(see Figs 65.2A to D).5,66,67 Mid-LV hypertrophy may be
recognized when the extent of the hypertrophy is
greatest at the level of the papillary muscles. The
highest midventricular gradients are usually recorded
at the level of the hypertrophied papillary muscles.
The triad of mid-LV hypertrophy, small LV cavity, and
hypertrophic papillary muscles predispose obstruction
as the LV comes into apposition with opposite wall
and the papillary muscles.66 Mid-LV obstruction may
occur on its own without development of an apical
akinetic chamber; many investigators believe that mid-LV
obstruction leads over time to apical aneurysm through
the mechanisms of afterload mismatch and supply
demand ischemia, ballooning, scar formation, and
thinning (see Figs 65.12 and 65.13). Contrast imaging is
helpful for demonstrating the obstruction together with
the secondary apical chamber.68 An invaluable clue in
diagnosing midventricular HCM and an apical akinetic
chamber is the detection of diastolic paradoxical jet flow
(Fig. 65.13).69 The jet is paradoxical because it originates
from the apex in early diastole and travels backward
toward the LV chambernormally diastolic flow travels
toward the apex from the mitral valve. As with apical HCM,
contrast is essential for diagnosis and should be given in
every case, and CMR is also useful for the detection of the
apical chamber.
1357
Fig. 65.12: Two hypertrophic cardiomyopathy (HCM) patients with midleft ventricle (LV) obstruction and apical akinetic
chambers. In both cases, diastole is on the left and systole is on the right. The thin long arrows show the mid-LV hypertrophy.
The arrowheads show the mid-LV obstruction. The thicker long arrow shows the apical akinetic chambers. In the patient below,
echocardiographic contrast has been given to enhance visualization of the apical akinetic chamber. (LA: Left atrium; LV: Left ventricle).
Source: Reproduced with permission from Shah A et al. Severe symptoms in mid and apical hypertrophic cardiomyopathy.
Echocardiography. 2009;26:92233.
1358
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 65.14A and B: (A) The Lobster Claw Abnormality is the midsystolic drop in left ventricle (LV) ejection velocities, here seen with
pulsed Doppler echocardiography at the entrance to the left ventricular outflow tract (LVOT). The midsystolic drop is due to the sudden imposition of afterload from mitralseptal contact. It is the cause of the midsystolic closure of the aortic valve and the spike and
dome pattern seen on aortic pressure tracings in patients with hypertrophic cardiomyopathy (HCM) and LVOT gradients > 60 mm Hg.
It is direct evidence of the corrosive effect of gradient in obstructive HCM; (B) The same patient after disopyramide and abolition of the
gradient. Note that the midsystolic drop in ejection velocities is no longer present. Note also the decrease in initial LV ejection acceleration, which is the mechanism of benefit of disopyramide. A decrease in ejection acceleration decreases the pushing force on the mitral
valve leaflets.
1359
DIFFERENTIAL DIAGNOSIS
Other etiologies that predispose to ventricular hypertrophy may be mistakenly diagnosed as HCM. This has
been termed pseudo-HCM. The most common etiology
of hypertrophy is chronic hypertension but others
include discrete subaortic membrane, athletes heart, LV
noncompaction, amyloidosis, AndersonFabry disease,
and Friedreichs ataxia.
Congenital subaortic membrane may cause high
gradients in the LVOT without the dynamic obstructive
patternthe Doppler spectra is similar to aortic stenosis
with midsystolic peak gradient and it is convex to the left.
In symptomatic patients, it is treated by surgical resection
of the membrane and replacement of the aortic valve if
there is more than mild aortic insufficiency.
LV noncompaction may superficially appear to be
hypertrophied myocardium. But left heart contrast echocardiography or CMR differentiates the compacted myocardium from the noncompacted, trabeculated myocardium.68
Thickened myocardium is the hallmark of cardiac
amyloidosis. The myocardium may have a ground glass
appearance, but this finding is not specific and can be
seen in renal failure and HCM. Moreover, with harmonic
imaging it is less prominent. A most valuable clue in making
the diagnosis of amyloidosis is the demonstration of low
voltage on the electrocardiogram, which is the reverse of any
other type of hypertrophy. Biopsy either from abdominal
fat, gastrointestinal tract, or the right ventricle makes this
diagnosis. The recent advent of transthyretin genetic analysis
is also useful in the rare patient with mutant transthyretin.
AndersonFabry disease, alpha-galactosidase A deficiency, a rare X-linked recessive lysosomal storage disease
may present with hypertrophy and infrequently with
SAM.78 It can be diagnosed with galactosidase levels in men,
and better by genetic testing in both genders. Treatment with
intravenous enzyme replacement is now available. Lastly,
Friedreichs ataxia is an autosomal recessive spinocerebellar
neuromyelopathy that may rarely present with severe hypertrophy in childhood, but more commonly presents as a
dilated cardiomyopathy.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 65.16A and B: Midsystolic drop in ejection velocities in midleft ventricle (LV) obstruction. (A) Pulsed wave (PW) spectral
Doppler with cursor located in the apical akinetic chamber at the neck of the midleft ventricular obstruction showing prominent
midsystolic drop in ejection velocity (thin arrow). There is an initial rise in velocities during the unobstructed phase (thick arrow),
followed by the marked decrease in midsystolic velocities and a second peak in early diastole. The drop in velocities corresponds
to the attenuation of the flow signal of the systolic jet in the obstructing neck during mid- and late systole; (B) Marked midsystolic
drop in LV ejection velocities in a patient with severe mid-LV obstruction. The pulsed Doppler is in the apical chamber at the
entrance of the neck of the mid-LV obstruction. Note the complete cessation of forward flow in this patient in midsystole and the
robust emptying of the chamber in late systole and early diastole. The midsystolic drop in ejection velocities is due to afterload
mismatch and provides persuasive evidence that the apical akinetic chamber is due to obstruction and supplydemand ischemia.
Source: Reproduced with permission from Sherrid M, et al. Reflections of inflections in hypertrophic cardiomyopathy. J Am Coll Cardiol.
2009;54:2129.
TREATMENT STRATEGIES IN
HYPERTROPHIC CARDIOMYOPATHY
Pharmacologic Therapy
Pharmacologic therapy is first-line treatment for symptomatic patients with obstructive HCM and adequate drug
trials should be administered before invasive measures
are contemplated. In patients with obstruction, the
pharmacologic treatment of HCM is based on negative
inotropes to relieve the dynamic obstruction by decreasing
anteriorly displacing drag forces on the mitral valve,
and thus potentiating normal posterior restraint of the
mitral apparatus by the papillary muscles and chordae.
Beta blockade is first-line negative inotropic therapy for
obstruction. Disopyramide in adequate dose is added
to patients who do not respond to beta blockade.83,84 The
goal of therapy in obstructed patients is improvement
in symptoms and functional status and reduction in
gradient. In patients without obstruction, beta blockers
and verapamil are used empirically for symptoms. The end
point here is improvement in functional status.
1361
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Figs 65.19A to D: Surgical separation of left ventricular inflow from outflow in obstructive hypertrophic cardiomyopathy (HCM):
extended myectomy and papillary muscle mobilization. (A) Line drawing of outflow relative to the mitral valve in early systole. Note
the anterior position of the mitral valve coaptation. The prominent midseptal bulge redirects outflow so that it comes from a relatively
posterior direction, catching the anteriorly positioned mitral valve and pushing it into the septum; (B) After subaortic septal resection.
The subaortic septum has been resected, but only down to the tips of the mitral leaflets. Flow is still redirected by the remaining septal
bulge so that it comes from a posterior direction. It still catches the mitral valve; SAM persists, as does obstruction; (C) The septal bulge
below the mitral leaflet tips has been resected, an extended myectomy. Now, flow tracks more anteriorly and medially, away from the
mitral leaflets; (D) Mobilization and partial excision of the papillary muscles is added to extended myectomy. The mitral coaptation plane
is now more posterior, explicitly out of the flow stream. (See Movie Clips 65.3 and 65.4).
Source: Reprinted with permission from Sherrid MV. Obstructive hypertrophic cardiomyopathy: echocardiography, pathophysiology, and
the continuing evolution of surgery for obstruction. Ann Thorac Surg. 2003;75:62032.
1363
ENDOCARDITIS PROPHYLAXIS
Endocarditis antibiotic prophylaxis is no longer
recommended for obstructive HCM. This is a relatively
rare complication of HCM with a prevalence of <1% that
mainly affects patients with resting obstruction.100 The
mitral valve apparatus and site of septal contact are most
common locations of vegetations. TEE is the modality of
choice to diagnose endocarditis and distinguish between
vegetations and redundant leaflets from HCM.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 65.21A to H: Variety of left and right ventricular structures at risk for alcohol-induced necrosis as detected by intraprocedural echocardiography. Opacification of the medial papillary muscle of the left ventricle (LV; A, arrow), the basal segment of the posterolateral wall
(B), the entire posterolateral wall in the apical four-chamber view (C, arrows), the entire interventricular septum (D), a papillary muscle
(arrow) of the right ventricle (RV) via a moderator band in association with a small contrast depot within the interventricular septum (E),
a small segment of the basal inferior portion (arrows) of LV free wall (parasternal short-axis view) (F), the entire posterolateral wall (G)
in the apical long-axis view (same patient as C; arrows), and a larger septal portion together with a right ventricular papillary muscle
(H, arrow). (LA: Left atrium; RA: Right atrium).
Source: Reproduced with permission from Faber L, et al. Targeting percutaneous transluminal septal ablation for hypertrophic obstructive cardiomyopathy by intraprocedural echocardiographic monitoring. J Am Soc Echocardiogr. 2000;13:10749.
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Cardiomyopathy: a report of the American College of
Cardiology Foundation/American Heart Association Task
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with the American Association for Thoracic Surgery,
American Society of Echocardiography, American Society
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Heart Rhythm Society, Society for Cardiovascular
Angiography and Interventions, and Society of Thoracic
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2. Bos JM, Towbin JA, Ackerman MJ. Diagnostic, prognostic,
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
36. Drinko JK, Nash PJ, Lever HM, et al. Safety of stress
testing in patients with hypertrophic cardiomyopathy. Am
J Cardiol. 2004;93(11):14434, A12.
37. Marwick TH, Nakatani S, Haluska B, et al. Provocation of
latent left ventricular outflow tract gradients with amyl
nitrite and exercise in hypertrophic cardiomyopathy. Am
J Cardiol. 1995;75(12):8059.
38. Klues HG, Leuner C, Kuhn H. Left ventricular outflow tract
obstruction in patients with hypertrophic cardiomyopathy:
increase in gradient after exercise. J Am Coll Cardiol.
1992;19(3):52733.
39. Gilligan DM, Nihoyannopoulos P, Fletcher A, et al.
Symptoms of hypertrophic cardiomyopathy, with special
emphasis on syncope and postprandial exacerbation of
symptoms. Clin Cardiol. 1996;19(5):3718.
40. Klues HG, Roberts WC, Maron BJ. Morphological
determinants of echocardiographic patterns of mitral
valve systolic anterior motion in obstructive hypertrophic
cardiomyopathy. Circulation. 1993;87(5):15709.
41. Klues HG, Roberts WC, Maron BJ. Anomalous insertion
of papillary muscle directly into anterior mitral leaflet in
hypertrophic cardiomyopathy. Significance in producing
left ventricular outflow obstruction. Circulation. 1991;
84(3):118897.
42. Haley JH, Sinak LJ, Tajik AJ, et al. Dynamic left ventricular
outflow tract obstruction in acute coronary syndromes: an
important cause of new systolic murmur and cardiogenic
shock. Mayo Clin Proc. 1999;74(9):9016.
43. Ohba Y, Takemoto M, Nakano M, et al. Takotsubo
cardiomyopathy with left ventricular outflow tract
obstruction. Int J Cardiol. 2006;107(1):1202.
44. Schwinger ME, OBrien F, Freedberg RS, et al. Dynamic
left ventricular outflow obstruction after aortic valve
replacement: a Doppler echocardiographic study. J Am
Soc Echocardiogr. 1990;3(3):2058.
45. Pearson AC, Gudipati CV, Labovitz AJ. Systolic and
diastolic flow abnormalities in elderly patients with
hypertensive hypertrophic cardiomyopathy. J Am Coll
Cardiol. 1988;12(4):98995.
46. Come PC, Bulkley BH, Goodman ZD, et al. Hypercontractile
cardiac states simulating hypertrophic cardiomyopathy.
Circulation. 1977;55(6):9018.
47. Hagge AA, Bruneval P, Levine RA, et al. The mitral valve
in hypertrophic cardiomyopathy: old versus new concepts.
J Cardiovasc Transl Res. 2011;4(6):75766.
48. Sasson Z, Yock PG, Hatle LK, et al. Doppler echocardiographic determination of the pressure gradient in
hypertrophic cardiomyopathy. J Am Coll Cardiol. 1988;
11(4):7526.
49. Schoendube FA, Klues HG, Reith S, et al. Long-term clinical
and echocardiographic follow-up after surgical correction
of hypertrophic obstructive cardiomyopathy with extended
myectomy and reconstruction of the subvalvular mitral
apparatus. Circulation. 1995;92(9 Suppl):II1227.
50. Kaple RK, Murphy RT, DiPaola LM, et al. Mitral
valve abnormalities in hypertrophic cardiomyopathy:
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65. Maron MS, Finley JJ, Bos JM, et al. Prevalence, clinical
significance, and natural history of left ventricular apical
aneurysms in hypertrophic cardiomyopathy. Circulation.
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66. Minami Y, Kajimoto K, Terajima Y, et al. Clinical implications
of midventricular obstruction in patients with hypertrophic
cardiomyopathy. J Am Coll Cardiol. 2011;57(23):234655.
67. Cecchi F, Olivotto I, Nistri S, et al. Midventricular
obstruction and clinical decision-making in obstructive
hypertrophic cardiomyopathy. Herz. 2006; 31(9):8716.
68. Comella A, Magnacca M. Pseudo-left ventricle apical
hypertrophy: bedside diagnosis with SonoVue contrast.
Echocardiography. 2004;21(6):5634.
69. Nakamura T, Matsubara K, Furukawa K, et al. Diastolic
paradoxic jet flow in patients with hypertrophic
cardiomyopathy: evidence of concealed apical asynergy
with cavity obliteration. J Am Coll Cardiol. 1992;19(3):
51624.
70. Harris KM, Spirito P, Maron MS, et al. Prevalence, clinical
profile, and significance of left ventricular remodeling
in the end-stage phase of hypertrophic cardiomyopathy.
Circulation. 2006;114(3):21625.
71. Barac I, Upadya S, Pilchik R, et al. Effect of obstruction
on longitudinal left ventricular shortening in hypertrophic
cardiomyopathy. J Am Coll Cardiol. 2007;49(11):120311.
72. Breithardt OA, Beer G, Stolle B, et al. Mid systolic septal
deceleration in hypertrophic cardiomyopathy: clinical
value and insights into the pathophysiology of outflow
tract obstruction by tissue Doppler echocardiography.
Heart. 2005;91(3):37980.
73. Sherrid MV, Gunsburg DZ, Pearle G. Mid-systolic drop in
left ventricular ejection velocity in obstructive hypertrophic
cardiomyopathythe lobster claw abnormality. J Am Soc
Echocardiogr. 1997;10(7):70712.
74. Conklin HM, Huang X, Davies CH, et al. Biphasic left
ventricular outflow and its mechanism in hypertrophic
obstructive cardiomyopathy. J Am Soc Echocardiogr.
2004;17(4):37583.
75. Maron BJ, Gottdiener JS, Arce J, et al. Dynamic subaortic
obstruction in hypertrophic cardiomyopathy: analysis by
pulsed Doppler echocardiography. J Am Coll Cardiol. 1985;
6(1):118.
76. Kirschner E, Berger M, Goldberg E. Hypertrophic obstructive
cardiomyopathy presenting with profound hypotension.
Role of two-dimensional and Doppler echocardiography
in diagnosis and management. Chest. 1992;101(3):71114.
77. Sherrid MV, Balaran SK, Korzeniecki E, et al. Reversal
of acute systolic dysfunction and cardiogenic shock
in hypertrophic cardiomyopathy by surgical relief of
obstruction. Echocardiography. 2011;28(9):E1749.
78. Sachdev B, Takenaka T, Teraguchi H, et al. Prevalence
of Anderson-Fabry disease in male patients with
late onset hypertrophic cardiomyopathy. Circulation.
2002;105(12):140711.
79. Maron BJ, Pelliccia A. The heart of trained athletes: cardiac
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Circulation. 2006;114(15):163344.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
CHAPTER 66
Echocardiographic Assessment of
Nonobstructive Cardiomyopathies
Rohit Gokhale, Manreet Basra, Victor Vacanti, Steven J Horn, Aylin Sungur, Robert P Gatewood Jr, Navin C Nanda
Snapshot
Cardiomyopathies
CARDIOMYOPATHIES
The diagnosis of a cardiomyopathy encompasses a wide
spectrum of cardiac diseases, all of which are characterized
by cardiac myocyte dysfunction, and therefore a myopathy.
While systolic dysfunction impairs end-organ perfusion,
diastolic dysfunction disrupts the perfusion to the cardiac
myocyte itself. This results in the propagation of a vicious
cycle whereby systolic dysfunction can ensue.
The World Health Organization (WHO) defined cardiomyopathy as a disease of the myocardium associated
with cardiac dysfunction in 1995.1 The cardiomyopathies
were classified according to anatomy and physiology
into five major types. The inclusion of arrhythmogenic
right ventricular cardiomyopathy/dysplasia (ARVC/D)
and primary restrictive cardiomyopathy (RCM) in the
classification for the first time broadened the definition,
characterizing the breadth of its presentation:
1. Dilated cardiomyopathy (DCM)
a. Idiopathic
b. Familial/genetic
c. Viral
Restricve Cardiomyopathy
d. Immune-mediated
e. Toxic/alcoholic
2. Hypertrophic cardiomyopathy (HCM)
3. RCM
4. ARVC/D
5. Unclassified cardiomyopathiesfor example, left
ventricle (LV) noncompaction
It also included specific cardiomyopathies such as the
following:
Ischemic
Valvular
Hypertensive
Inflammatory: Idiopathic, autoimmune, and infectious
Metabolic: Endocrine-related; storage diseases hemochromatosis/glycogen storage
Muscular dystrophies: Duchenne, Becker, and myotonic
Peripartum
In 2006, the American Heart Association (AHA)
scientific statement proposed a more contemporary definition and classification of the cardiomyopathies.2
1370
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1371
Figs 66.1A to D: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A), parasternal
short-axis (B), and apical four-chamber (C) views showing marked global left ventricle (LV) hypokinesis. Arrow points to a catheter in
the right heart. The echogenic tricuspid valve (TV) annulus probably represents fatty infiltration; (D) M-mode also showing poor LV function with increased mitral E point to interventricular septum separation. (Ao: Aorta; DA: Descending thoracic aorta; IVS: Interventricular
septum; LA: Left atrium; MV: Mitral valve; PW: Posterior wall; RV: Right ventricle) (Movie clips 66.1A to C).
1372
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 66.2A to D
1373
Figs 66.2A to E: Dilated cardiomyopathy. Live/real time threedimensional transthoracic echocardiography (3D TTE). (A to C)
Systematic and sequential anteroposterior cropping of right ventricle
(RV) demonstrates the moderator band (arrowhead) as well as other
trabeculations and papillary muscles (arrow) in the right ventricle
(RV); (D and E) Further cropping shows only a few trabeculations in
the RV apex. There is no evidence of noncompaction. Also, there is
no evidence of a mass or clot in the RV apex, which was suspected
on the two-dimensional (2D) study. A prominent trabeculation in the
RV apex very clearly delineated by 3D TTE (arrows in the Movie
clip 66.2, Part 1) was most likely the culprit for the mass-like lesion
suspected on the 2D echocardiogram. Both ventricles are dilated and
show globally poor motion typical of dilated cardiomyopathy. Arrow in
A, B, D, and E points to a false tendon in left ventricle (LV). Movie clip
66.2, Part 2 shows another patient with dilated cardiomyopathy and
globally poor biventricular function. The arrowhead shows a large
clot in the LV which when further cropped demonstrates a central
echolucency consistent with clot lysis. A few trabeculations are also
seen. (LA: Left atrium; RA: Right atrium) (Movie clips 66.2 Parts 1
and 2). Source: Reproduced with permission from Bodiwala K, et
al. Live three-dimensional transthoracic echocardiographic assessment of ventricular noncompaction. Echocardiography. 2005;22:
61120.
Figs 66.3A and B: Dilated cardiomyopathy. (A) The left ventricle (LV) end-diastolic dimension in the parasternal long axis has decreased
in the follow-up study (right side) as compared to baseline examination (left side). Movie clips 66.3A (parasternal long-axis view), 3B
(parasternal short-axis view), and 3C (apical four-chamber view) also show improved LV function 1 year after antifailure regimen.
(Ao: Aorta; DA: Descending aorta; LA: Left atrium; PW: Posterior wall; RA: Right atrium; RV: Right ventricle; VS: Ventricular septum).
1374
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 66.4A and B: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A) and apical fourchamber; (B) views demonstrate LV and left atrial (LA) dilatation. (RA: Right atrium; RV: Right ventricle).
Evidence of Effusion
Pericardial and pleural effusions are readily imaged
on echocardiography and can be signs of significant
1375
Figs 66.6A and B: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Mitral valve M-mode echocardiography
showing a B bump (A) predictive of significant left ventricular end-diastolic pressure (LVEDP) and an enlarged E-point septal separation
(EPSS; B) in a patient with dilated cardiomyopathy due to a reduced stroke volume with poor systolic function. A normal EPSS should
generally be < 1 cm.
Doppler Echocardiography
Doppler echocardiography assists in assessing cardiac
output, cardiac index, stroke volume, stroke volume index
(stroke volume indexed to body surface area), ventricular
diastolic function, pulmonary arterial pressures, and right
atrial pressure. The product of the transaortic velocity time
integral with the cross sectional area of the left ventricular
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Additional Findings
With long-standing DCM, the mitral regurgitation and
elevated left ventricular diastolic pressures result in left
atrial enlargement. This in turn predispose patients to
atrial fibrillation, which can result in further decrement
of mechanical atrial function. With slow flow within the
atrium, spontaneous echocardiographic contrast can
be noted. Transesophageal echocardiography can often
demonstrate spontaneous echocardiographic contrast
within the left atrium indicative of poor systolic function
among patients with atrial fibrillation. Sometimes,
spontaneous echocardiographic contrast may be also
seen in the LV. Although mural thrombi in the LV are more
frequent in ischemic cardiomyopathy with significant wall
motion abnormalities, they can occur in nonischemic
DCM. They may be laminar, pedunculated, or mobile
based on the duration of onset of systolic dysfunction
(Figs 66.8 and 66.9; Movie clips 66.8A and B; 66.9Part 1
and 2).14
1377
Figs 66.8A and B: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Apical four- (A) and three- (B) chamber
views. Spontaneous echo contrast (arrowhead) is noted within the left ventricle (LV) cavity in B. Arrow points to a pacemaker in the right
heart. (Ao: Aorta; DA: Descending aorta; LA: Left atrium; RA: Right atrium; RV: Right ventricle) (Movie clips 66.8A and B).
Figs 66.9A and B: Multiple thrombi in a patient with poor ventricular function. Two-dimensional transthoracic echocardiography. The
movie clip on day 1 shows prominent thrombi (arrows), some of them mobile, in both atria and in the left ventricle (LV). A few show
central echolucencies of varying sizes consistent with clot lysis. Repeat examination on day 2 shows the clots to be smaller and less
prominent, most likely related to significant resorption. There was no clinical evidence for embolization. A and B are representative
frames from the movie clip. (Ao: Aorta; LA: Left atrium; RA: Right atrium; RV: Right atrium) (Movie clips 66.9 Parts 1 and 2).
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 66.10A to F
1379
Figs 66.10A to G: Biventricular pacing. Two-dimensional transthoracic echocardiography. Before removal of coronary sinus lead.
Parasternal long-axis (A), short-axis (B), apical four-chamber
(C), and subcostal (D) views. Left ventricle (LV) shows mild to
moderate dysfunction with an estimated ejection fraction of 37%.
The arrow points to a pacemaker lead in the right ventricle (RV).
RV function is normal. After removal of coronary sinus lead due to
symptomatic diaphragmatic stimulation. Parasternal long-axis (E),
short-axis (F), and apical four-chamber (G) views show the marked
deterioration of LV function with an estimated ejection fraction of
1015%. RV function is unchanged. (Ao: Aorta; DA: Descending
thoracic aorta; L: Liver; LA: Left atrium; MV: Mitral valve; RA: Right
atrium) (Movie clips 66.10A to G).
ECHOCARDIOGRAPHY IN ASSESSING
VENTRICULAR REMODELING
Association between increased LV diameters at baseline
and poor outcome after CRT implant has been described
in some studies.18,19 LV diameter measurements by
M-mode echocardiography allow acceptable estimation
of LVEF and correlate with LV volumes, but are hindered
by a wide margin of error when it comes to accurate LV
size assessment, especially for enlarged ventricles.20 The
addition of parameters such as sphericity index and endsystolic volume enable a more valid and reliable estimate
of the extent of LV dilatation.
Patients with extensive remodeling [end-systolic
volume index (ESVI) > 103 mL/m2] and only one parameter of intraventricular dyssynchrony at baseline
showed no significant changes in ejection fraction after
CRT.21 Therefore, patients with low likelihood to improve
in LV function after CRT were more likely to be those with
larger LVs. Large ESVI at the time of implant was also an
independent and strong predictor of poor outcome during
long-term follow-up (Figs 66.11A and B and Movie clip
66.11).
Furthermore, increased end-systolic volume limits
improvement in LVEF after revascularization in patients
with chronic ischemic cardiomyopathy, despite presence
of viability.22 Therefore, it has also been proposed that
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Figs 66.11A and B: Cardiac resynchronization therapy (CRT). Biventricular pacing. Two-dimensional transthoracic echocardiography.
Apical four-chamber view. (A) Patient with cardiomyopathy with poor left ventricle (LV) ejection fraction; (B) Marked improvement in LV
function is noted following biventricular pacing. Arrowhead points to a pacing wire. (LA: Left atrium; MV: Mitral valve; RA: Right atrium;
RV, right ventricle). These are representative frames from the movie clip (Movie clip 66.11).
Figs 66.12A and B: Left ventricular assist device in a patient with cardiomyopathy. Two-dimensional transthoracic echocardiography.
Parasternal long-axis view. (A) Note very poor function of both ventricles. The aortic valve (arrow) does not open and remains in the
closed position throughout because all the ventricular flow is routed by the left ventricle (LV) assist device into the proximal ascending
aorta (Ao). There is no direct ejection of flow into the AO through the aortic valve; (B) Color Doppler examination shows significant
aortic regurgitation (AR). (LA: Left atrium; RV: Right ventricle) (Movie clips 66.12A and B).
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Peripartum
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 66.14C to H
Figs 66.14I to N
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 66.14A to R: Peripartum cardiomyopathy. Morphological assessment of left ventricular thrombus by live three-dimensional transthoracic echocardiography. (A) Arrowhead points to the thrombus attached to left ventricle (LV) apex; (B and C) Transverse plane
(TP, horizontal plane or short axis) section at the attachment point of the thrombus (arrowhead) shows it to be highly echogenic (viewed
en-face, arrow in C); (D and E) TP and longitudinal plane (LP, vertical plane or long axis) sections through the thrombus (arrowhead)
showing the echogenic attachment (arrow) and a large echolucency within the thrombus consistent with lysis; (F to H) TP and both
TP and LP sections at midthrombus level showing clot lysis. (I and J) TP section at thrombus tip level showing a solid rim (viewed
en-face); (K and L) LP section through thrombus showing clot lysis. (M and N) Frontal plane (FP) section through the thrombus viewed
en-face (M) and after tilting (N); (O) TP and LP sections through the thrombus. The data set has been tilted and rotated to show the
position of the FP; (P to R) Oblique sections through the thrombus. The arrow in R points to residual fibrin strands within the lytic area
of the thrombus. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle) (Movie clip 66.14). Source: Reproduced with
permission from Sinha A, et al. Morphological assessment of left ventricular thrombus by live three-dimensional transthoracic echocardiography. Echocardiography. 2004;21:64955.
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Figs 66.16A to E: Left ventricular noncompaction. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A),
apical three-chamber (B), apical four-chamber (C and D) views
showing the trabeculations of the LV wall and deep intertrabecular
recesses (arrowheads) characteristic of left ventricle (LV)
noncompaction. (E) Apical two-chamber view with color Doppler
showing direct blood flow from the ventricular cavity into deep
intertrabecular recesses in LV noncompaction. (Ao: Aorta; LA: Left
atrium; RA: Right atrium; RV: Right ventricle).
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Figs 66.17A and B: Left ventricular noncompaction. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A) and
apical two-chamber (B) views with Doppler evidence of flow in the recesses (B). (Ao: Aorta; LA: Left atrium; LV: Left ventricle; MR: Mitral
regurgitation; MV: Mitral valve; RV: Right ventricle) (Movie clips 66.17A and B).
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Takotsubo Cardiomyopathy/Left
Ventricle Apical Ballooning Syndrome
Takotsubo cardiomyopathy, also called stress-induced
cardiomyopathy or broken heart syndrome, is an
increasingly reported syndrome characterized by transient
systolic dysfunction of the apical and/or mid segments
of the LV that occurs in the setting of acute emotional
stress. The initial presentation often mimics that of an
acute MI with concomitant rise in cardiac biomarkers,
electrocardiographic abnormalities, and in severe
cases with features of acute CHF.43 However, coronary
angiography does not reveal a significant obstruction
that can explain the nature or magnitude of systolic
dysfunction noted. The etiology is thought to be related to
the hyperadrenergic state associated with emotional stress
or psychological trauma.
The classical finding of apical ballooning (typical
variant) and/or midventricular hypokinesis is usually
seen on left ventriculography or echocardiography.4345 In
a minority of cases, the transient left ventricular hypokinesis
is restricted to the midventricular segments (atypical
variant or apical sparing variant) without involvement
of the apex.46 As reported by Kurowski et al. the atypical
variant may account for nearly 40% of patients with
Takotsubo cardiomyopathy.46 More recently, Manzanal
et al.47 also described a case series of patients with
the atypical variant, the so-called inverted Takotsubo
Tachycardia-Induced Cardiomyopathy
and Other High-Output States
Tachycardia-induced cardiomyopathy is a well-recognized
entity leading to DCM, but its exact incidence is still
unknown. It should be suspected in patients with atrial as
well as ventricular arrhythmias including atrial fibrillation,
atrial tachycardias, re-entrant arrhythmias, ventricular
tachycardias, and premature ventricular contractions
(PVCs) resulting in persistently high heart rates.
It has all the features of DCM on echocardiography
with the exception that LV end-diastolic dimension (EDD)
tends to be smaller in patients with tachycardia-mediated
cardiomyopathy.49 Follow-up of the ventricular function
after a period of 36 months with satisfactory rate control
will often demonstrate significant recovery of function
(Fig. 66.27A and B; Movie clips 27A and B).
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Figs 66.19A to E: Combined left and right ventricular noncompaction. Live/real time three-dimensional transthoracic echocardiography. (A and B) Arrows point to prominent trabeculations in
both ventricles; (C) Arrows show multiple prominent trabeculations
in RV; (D) Transverse cropping of left ventricle (LV) apical
area shows a honeycomb-like appearance (arrow) typical of
noncompaction; (E) Echo contrast study using perflutren lipid
microspheres shows filling of intertrabecular recesses with
the contrast agent (arrows). See Movie clip 66.19, Part 1 to 5.
Systemic cropping of the three-dimensional data set demonstrates
extensive trabecular involvement of the LV (arrowheads). (AV:
Aortic valve; LA: Left atrium; LV: Left ventricle; MV: Mitral valve;
RA: Right atrium; RV: Right ventricle) (Movie clips 66.19 Parts 1
to 5). Source: Reproduced with permission from Bodiwala K, et al.
Live three-dimensional transthoracic echocardiographic assessment of ventricular noncompaction. Echocardiography. 2005;
22:61120.
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Figs 66.20A to F
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Figs 66.22A to C: Isolated right ventricular noncompaction. Twodimensional and live/real time three-dimensional transthoracic
echocardiography. (A) Two-dimensional study. Arrowheads point
to a few muscle bands in the right ventricular apex but there is
no clear-cut evidence for noncompaction; (B) Three-dimensional
study. Cropping of the image reveals a honeycomb-like appearance typical of RV noncompaction. Trabeculations (arrowhead) fill
the distal 40% of RV almost completely; (C) Intracardiac echocardiography. Shows RV noncompaction (arrowhead). (LA: Left atrium;
LV: Left ventricle; RA: Right atrium; RV: Right ventricle) (Movie
clips 66.22A to C).
Source: Reproduced with permission from Reddy VK, et al.
Incremental value of live/real time three dimensional transthoracic
echocardiography in the assessment of right ventricular masses.
Echocardiography. 2009;26:598609.
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B
Figs 66.26A and B: Takotsubo cardiomyopathy. Apical four-chamber view. Velocity vector imaging. The individual arrows point
to the direction of endocardial motion, while the lengths are proportional to velocity. (A) Compared to baseline (left figure), the
velocity of motion has significantly increased in the left ventricle (LV) mid segments, while the apex is still hypokinetic during follow
up (right figure); (B) No change is noted by visual inspection in the left atrial (LA) wall motion during follow-up (right figure) as compared to baseline (left figure). This patient belonged to a series of five patients with Takotsubo cardiomyopathy, in whom the left
atrium also appeared to be involved by velocity vector imaging with a statistical tendency toward improvement during follow-up.
(RA: Right atrium; RV: Right ventricle). These are representative frames from the movie clips. (Movie clips 66.26A and B).
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Figs 66.27A and B: Tachycardia-induced cardiomyopathy. Two-dimensional transthoracic echocardiography. (A) Parasternal longaxis view. The left ventricle (LV) is dilated with poor function. The heart rate was 133/min; (B) Apical four-chamber view. The patient was
placed on a -blocker, which resulted in heart rate reduction and improvement in LV function. (Ao: Aorta; DA: Descending aorta; LA: Left
atrium; MV: Mitral valve; RA: Right atrium; RV: Right ventricle) (Movie clips 66.27A and B).
Figs 66.28A and B: Ischemic cardiomyopathy. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A) and apical
four-chamber (B) views. All the chambers are markedly dilated with extremely poor function of both ventricles. The septum is thin and
echogenic, consistent with scarring and fibrosis indicating ischemic heart disease. Arrowhead points to a pacemaker wire. (Ao: Aorta;
LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RA: Right atrium; RV: Right ventricle) (Movie clips 66.28A and B).
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Toxic Cardiomyopathies
Fig. 66.29: Arrhythmogenic right ventricular dysplasia. Twodimensional transthoracic echocardiography. Apical four-chamber
view shows diminished right ventricular (RV) function as well
as presence of small, localized berry-like aneurysms involving
the free wall (arrows) typical of this entity. The left ventricle (LV)
function is normal. (LA: Left atrium; MV: Mitral valve; RA: Right
atrium) (Movie clip 66.29A).
Chemotherapy-Induced Cardiomyopathy
Chemotherapy with adriamycin or other anthracyclines is
often adopted for a variety of hematological malignancies.
It, however, is associated with cardiotoxicity and can be
an issue among adult patients receiving chemotherapy
as well as survivors of childhood cancers.62 The toxicity is
likely due to a combination of factors including reactive
oxygen species generation and myocardial cellular
toxicity due to adriamycin itself. While acute toxicity can
result in cardiomyopathy that can in part be reversed
by discontinuation of the chemotherapy, late onset of a
cardiomyopathy is irreversible.
Late onset chronic progressive cardiomyopathy occurs
more than 1 year after anthracycline treatment.63,64 With
the loss of cardiomyocytes, cardiac function begins to
deteriorate with resultant LV wall thinning and in some
cases, progressive LV dilation.65,66
Echocardiographic abnormalities may include
decreased LV fractional shortening, end-diastolic posterior
wall thickness, and mass. Associated would be decreased
LV contractility and increased LV afterload. Left ventricular
dimension may be increased, normal, or decreased
(Figs 66.30A and B; Movie clip 66.30). Strain rate imaging
is a new technique to evaluate myocardial mechanics
and can detect early changes prior to a decrement in
the ejection fraction. This technique will enable early
detection of patients at risk of cardiomyopathy and would
be useful in managing chemotherapy regimens.
Although adults typically develop chronic DCM
after anthracycline chemotherapy, children at the end
of anthracycline treatment have a DCM, which may
then progress to a RCM.66 Afterload increases, in spite of
normal-to-reduced blood pressure caused by decreased
LV wall thickness and mass. Therefore, the elevated LV
afterload reduces the cardiac output to a greater extent
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Figs 66.30A and B: Adriamycin-induced cardiomyopathy. Two-dimensional transesophageal echocardiography. Parasternal long (A)
and short; (B) axis views. Left ventricle (LV) is markedly enlarged and severely hypokinetic. The right ventricle (RV) is also very hypokinetic. Arrow points to a catheter in the right heart. (LA: Left atrium; RA: Right atrium) (Movie clip 66.30).
Fig. 66.31: Dilated cardiomyopathy. Two-dimensional transthoracic echocardiography. Left ventricle (LV) short-axis view at the
level of the papillary muscles shows decreased rotation of the
ventricle in systole and unrotation in diastole. This is a representative frame from the movie clip demonstrating a dilated LV.
(RV: Right ventricle). (Movie clip 66.31).
Alcohol-Induced Cardiomyopathy
Alcohol-induced cardiomyopathy (ACM) is among the
leading causes of nonischemic DCM in the United States.67
RESTRICTIVE CARDIOMYOPATHY
The distinct morphological and hemodynamic characteristics that separate restrictive from the more common
dilated and hypertrophic cardiomyopathies are:
1. Nondilated ventricle (<40 mL/m2) with normal wall
thickness (<11.2 cm)
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Doppler Findings
Evaluation by Doppler echocardiography is crucial to
the diagnosis of RCM. With advanced disease, often an
elevated peak mitral inflow velocity, rapid early mitral
inflow deceleration and reduced early annular velocity
on tissue Doppler imaging is noted. Pertinent values to be
considered in the diagnosis include:
1. The E/A ratio of mitral valve inflow is pathologically
elevated typically > 2.070 and the deceleration time is
shortened to < 160 milliseconds.
2. Doppler tissue imaging of the mitral annulus also
reveals abnormally low diastolic Doppler annular
velocities typically < 10 cm/s.
3. The high mitral inflow velocities with low annular
velocity also result in a particularly high E/E' ratio > 25.
4. Since restrictive cardiomyopathies are usually global,
the RV may also be affected. Hypertrophy can be seen
in the right ventricle due to pressure overload and also
abnormal tricuspid valve inflow velocities. These can
lead to abnormal hepatic vein flow as well.
The many different etiologies of RCM also show
characteristic patterns on echocardiography.
Amyloid Cardiomyopathy
The annual incidence of systemic amyloidosis is approximately 610 cases per million of the general population.
Evidence suggests that the incidence of cardiac amyloidosis is increasing as the mean life expectancy rises. This
may also be due, in part, to improvements in diagnostic
modalities that have led to earlier identification of this
condition.71
The development of a cardiomyopathy results from the
deposition of amyloid within the myocardium. It presents
predominantly as a RCM characterized initially by diastolic
dysfunction, progressing to profound biventricular systolic
dysfunction and arrhythmias. The two most common forms
involve deposition of monoclonal light chain fragments
in the myocardium (termed primary amyloidosisAL),
or fragments of serum amyloid in secondary amyloidosis
(secondary amyloidosisAA). Endomyocardial biopsy
shows interstitial prominence with massive amyloid
deposits and varying size myocardial cells often containing
vacuoles.
In cardiac amyloidosis, echocardiography demonstrates symmetric left ventricular wall thickening typically
involving the interventricular septum, small ventricular
chambers, thickening of the atrial septum, pericardial
effusion, and dilated atria.72,73 Right ventricular diastolic
dysfunction may also be present in association with
increased right ventricular wall thickness.73 The typical
findings of biventricular wall thickness usually precede
multichamber dilation seen in the later stages. Intracardiac
thrombi may be present in up to 35% of patients with the
AL type and up to 15% in other types74 of amyloidosis
(Figs 66.32A to C; Movie clips 66.32A and B).
With progression of amyloidosis, decreased systolic
thickening of the ventricular septum and globally
decreased LVEF ensues. The ventricular walls show a
granular and brightly reflective sparkling appearance,
corresponding to amyloid particle deposition (Figs 66.33A
to H; Movie clip 66.33).
The cardiac valves typically have a thickened
appearance and although their movement is usually
normal, valvular regurgitant flow is often detected.72
Interestingly, it has been suggested that the characteristic
speckle pattern might not be as obvious when using
harmonics on later-generation echocardiography
machines.71 The use of echocardiography to differentiate
cardiac constriction from RCM, while not definitive,
is certainly helpful. The measure of the transvalvular
diastolic velocities across the atrioventricular valves is
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A
Figs 66.33A and B
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 66.33A to H: Amyloidosis. Two-dimensional transthoracic echocardiography. Both the ventricular septum (IVS) and left ventricle
(LV) posterior wall (PW) are echogenic and markedly thickened because of amyloid deposition, not hypertrophy. These are visualized
in parasternal long- (A systole, B diastole) and short- (C systole, D diastole) axis as well as apical four-chamber (E systole, F diastole)
views. LV function is diminished; (G) M-mode examination showing similar findings; (H) Tissue Doppler imaging of the medial mitral
annulus shows a low E' velocity of 3.8 cm/s (arrow) consistent with poor longitudinal shortening of LV. (Ao: Aorta; DA: Descending aorta;
MV: Mitral valve; RA: Right atrium; RV: Right ventricle; RVW: Right ventricular wall) (Movie clips 66.33A to C).
Fabry Disease
Fabry disease (FD) is a lysosomal storage disease,
caused by mutations in the gene encoding the enzyme
-galactosidase A, resulting in a deficit in enzyme activity.
It is X-linked and is characterized by the progressive
accumulation of glycosphingolipids. Multiple organs can
be affected with the heart, kidneys, and the neurological
system in particular being common targets.75
Cardiac involvement is quite frequent and is one of the
main causes of death in patients with advanced FD. In the
heart, glycosphingolipids deposition causes progressive
left ventricular hypertrophy (LVH) that can at times mimic
the morphological and clinical characteristics of HCM.76
Fig. 66.34: Fabry disease. Two-dimensional transthoracic echocardiography. Arrow shows an echolucency within the myocardium
of the ventricular septum, which has been reported in patients with
this entity. This finding reflects glycosphingolipids compartmentalization in which the subendocardial layer is spared resulting in
an echolucent area. Note that left ventricle (LV) function is poor.
Arrowhead in the Movie clip 66.34 points to an intracardiac defibrillator lead. (LA: Left atrium; RA: Right atrium; RV: Right ventricle).
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Hypereosinophilic Cardiomyopathy
The WHO published a recent update that outlined the
diagnosis of hypereosinophilic syndrome (HES).84 These
conditions are characterized by:
1. Persistent marked eosinophilia (>1,500 eosinophils/
mm3);
2. The absence of a primary cause of eosinophilia (such
as parasitic or allergic disease); and
3. Evidence of eosinophil-mediated end organ damage.
Cardiac involvement unrelated to hypertension,
atherosclerosis, or rheumatic disease is identified in 20%
of patients (only 6% at the time of initial presentation).85
Typical cardiac findings include endocardial fibrosis and
mural thrombus, which is most frequent in the apices of
both ventricles and is also characterized by progressive
heart failure.
The pathophysiology involves eosinophil infiltration
of cardiac tissue and release of toxic mediators resulting
in endocardial damage and formation of platelet thrombi.
These mural thrombi pose a high risk for embolization.
Later with disease progression, there is fibrous thickening
of the endocardial lining leading to a RCM.86,87
Hence, three stages of pathophysiologynecrosis,
thrombosis, and fibrosis are usually identified. Endomyocardial fibrosis (EMF), which was initially described in
1936 by Loeffler, is the most characteristic cardiovascular
abnormality.88 Valvular insufficiency can result from mural
endocardial thrombosis and fibrosis involving leaflets of
the mitral or tricuspid valves.89,90 In addition, entrapment
Findings on Transthoracic
Echocardiography
Echocardiographic evaluation during the necrosis stage
is usually normal. The hallmark echocardiographic
finding is the persistent obliteration of the apex of the
left or right ventricle, or both, by laminar thrombus.91 The
common differential diagnosis of LV apical infiltration and
obliteration include LV apical thrombus and apical HCM,
which can be differentiated by their echocardiographic
characteristics. A LV apical thrombus is associated with
underlying LV dysfunction and wall motion abnormality. In
apical cardiomyopathy, the LV apex is visualized in diastole
with obliteration in systole, thus producing the peculiar
ace of spades configuration. HES, however, shows
persistent apical obliteration, mitral valve involvement,
and progressive features of a RCM. Resolution of the
apical infiltration may sometimes be observed if treated
appropriately by medical and surgical intervention.
Tricuspid and pulmonary valvular thickening may
accompany right ventricular involvement with thrombus
formation in the RV apex. Doppler evaluation of the valves
enables the estimation of valvular regurgitation and the
determination of restrictive physiology (Figs 66.35A to H;
Movie clips 66.35A to E).
Contrast Echocardiography
Contrast echocardiography may be invaluable in the
diagnosis of hypereosinophilic cardiomyopathy enabling
it to be differentiated from a thrombus or apical HCM.
Specifically since it delineates the shape of LV, it
distinguishes between hypertrophy and complete persistent obliteration in these conditions.92
Transesophageal Echocardiography
When the transthoracic echo images are poor or limited,
transesophageal echocardiography is useful. The deep
transgastric views enable visualization of the LV apex and
can demonstrate the obliteration of the LV cavity thus
confirming the diagnosis.
Endomyocardial Fibrosis
In the third stage of HES, significant EMF ensues, resulting
in a RCM. It is considered a particularly devastating
Figs 66.35A to F
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Figs 66.35A to H: Loeffler endocarditis. Two-dimensional transthoracic echocardiography. Baseline. (A and B) Modified four-chamber
view. (A) Arrowhead shows marked thickening of the tricuspid valve (TV). The arrow points to a large mass in the right ventricular
(RV) apex. A small pericardial effusion (PE) is also noted; (B) Color Doppler examination. Arrowhead shows moderate to severe TV
regurgitation; (C and D) Aortic (AO) short-axis view; (C) Shows marked thickening of the pulmonary valve (PV); (D) Color Dopplerguided continuous wave Doppler interrogation shows a peak gradient of 48.50 mm Hg consistent with moderate PV stenosis (arrow).
After therapy; (E and F) Modified four-chamber view. Show complete normalization of TV and RV apex. Arrowhead points to moderator
band. PE is absent; (G and H) Aortic short-axis view; (G) Thickening involving the PV has completely regressed and the valve appears
structurally normal (arrowhead); (H) Color Doppler-guided continuous wave Doppler examination shows normal flow velocities across
the PV (arrow). (LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery; RA: Right atrium) (Movie clips 66.35A to E).
Source: Reproduced with permission from Garg A, Nanda NC, Sungur A, et al. Transthoracic echocardiographic detection of pulmonary
valve involvement in Loefflers endocarditis. Echocardiography (2013, in press).
Fig. 66.37: Sarcoidosis. Two-dimensional transthoracic echocardiography. Parasternal long-axis view. Both the ventricular
septum (VS) and posterior wall (PW) are echogenic, consistent
with myocardial fibrosis. (Ao: Aorta; LA: Left atrium; MV: Mitral
valve; RV: Right ventricle) (Movie clip 66.37).
OTHER INFILTRATIVE
CARDIOMYOPATHIES
Sarcoidosis
Sarcoidosis is a systemic disease characterized by the
formation of noncaseating granulomas that can infiltrate
the myocardium. Cardiac involvement only occurs in
approximately 5% of patients with systemic sarcoidosis.
Myocardial granulomas with central areas displaying
low signal intensity characteristic of fibrosis and a high
peripheral signal intensity corresponding to edema are
typically seen on cardiac MRI.98
Patients with cardiac sarcoidosis may manifest with a
variety of clinical scenarios varying from cardiomyopathy
and heart failure to conduction system abnormalities and
ventricular tachyarrhythmias.
While contemporary diagnosis often entails the use
of cardiac MRI, traditional echocardiographic findings
are useful in identifying cardiac sarcoidosis. The usual
echocardiographic appearance is that of a DCM. The
ventricle may be globally hypokinetic or the patchy nature
of sarcoid infiltration of the heart may result in regional
wall motion abnormalities. With edema or infiltration,
mild wall thickening may also be present. This is noted
on echocardiography by the presence of bright shadows
consistent with infiltration.
Typically with progression, areas of wall thinning
are seen, most commonly in the ventricular septum,
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Hemochromatosis
Hemochromatosis represents an iron overload disorder
characterized by the accumulation of iron within various
cells, including cardiac myocytes. Cardiac manifestations
of hemochromatosis are characterized by systolic
dysfunction, and cardiac MRI can detect and quantify
myocardial iron infiltration using T2 weighted imaging.
Liver biopsy is the definitive test for iron overload.102 Serum
transferrin saturation is typically > 45% and elevated serum
ferritin levels are seen, which help confirm the diagnosis
of hemochromatosis.
Although cardiac MRI is a superior imaging modality
for the diagnosis of cardiac hemochromatosis, TTE is
useful for following disease response to chelation therapy
and/or phlebotomy. The echocardiographic features
of hemochromatosis include mild LV dilatation, LV
systolic dysfunction, normal wall thickness, and biatrial
enlargement.103 The degree of iron deposition in the
myocardium correlates with the degree of LV dysfunction.
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Figs 66.38A to C: Septicemic myocarditis. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A) and apical fourchamber (B) views. This patient with streptococcal pneumonia developed poor biventricular function consistent with myocarditis; (C) Apical
four-chamber view. Further deterioration of ventricular function was
noted on the next day and the patient succumbed in the next few days
from multiorgan failure. (Ao: Aorta; LA: Left atrium; LV: Left ventricle;
PW: Posterior wall; RA: Right atrium; RV: Right ventricle; VS: Ventricular septum) (Movie clips 66.38A to C).
Septic Cardiomyopathy
Cardiogenic Shock
LV function
Globally reduced
Often globally
reduced
LV dilatation
Mild
Severe
Mitral inflow
velocities
Mostly normal
Restrictive pattern
high E-wave velocities, low A-wave
velocities
RV
involvement
Often decreased
RV systolic function
and RV dilatation
Less common
Reversibility
Yes (days)
Depends on etiology
1407
The degree of global dysfunction in septic cardiomyopathy may be masked by severe peripheral vasodilatation causing significantly reduced afterload, thereby
appearing as if cardiac output is not significantly reduced.
However, once patients receive restoration of normal
afterload with fluid resuscitation and/or vasopressor
support, the degree of sepsis-induced LV dysfunction
is often unmasked. Once the diagnosis of septic
cardiomyopathy is made, serial bedside transthoracic
echocardiograms may be obtained to assess the degree of
LV and RV dysfunction in response to treatment.
HIV-Associated Cardiomyopathy
Metabolic Cardiomyopathy
Metabolic cardiomyopathies encompass a wide range
of inherited metabolic disorders and a wide spectrum
of other pathological conditions. Inherited metabolic
disorders often present in childhood and include defects
in mitochondrial long-chain fatty acid oxidation, carnitine
deficiency disorders, respiratory chain defects, disorders
Diabetic Cardiomyopathy
Two-Dimensional Echo
Echocardiography can be used to ascertain the systolic
and diastolic function. Diastolic dysfunction is depicted
by reduced early peak mitral inflow velocities at annular
septal and lateral levels in early diastole, as noted on
Doppler echo.
Increased myocardial reflectivity in patients with
diabetes-related heart disease has also been reported
(Figs 39A to C).114
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C
arising from the enterochromaffin cells most often in the
gastrointestinal tract. These tumors are malignant with
an incidence of around 1.22.1 per 100,000 of the general
population.115 They are characterized by the production
of vasoactive substances such as serotonin, 5-hydroxytryptamine, bradykinin, tachykinin, and prostaglandins,
which result in the typical clinical features of flushing,
diarrhea, bronchospasm, and hypotension.115,116
Carcinoid heart disease is seen in > 50% of patients with
carcinoid syndrome.117 In approximately 20% of patients,
carcinoid heart disease is the primary presentation of
metastatic carcinoid disease. Historically, the association
between carcinoid tumors and specific cardiac disease
was described in the 1950s.118 The echocardiographic
features of carcinoid heart disease were well described in
the 1980s and used reliably in its diagnosis.119,120
Cardiac involvement is predominantly right-sided as
the lungs filter the tumor products before they reach the
left atrium. It is characterized by plaque-like deposits of
fibrous tissue, typically on the endocardium of the right-
Echocardiographic Features
On 2D echocardiography, the tricuspid valve leaflets are
typically thickened and shortened. The leaflets become
increasingly retracted with progression of the disease,
resulting in reduced mobility. The septal and the anterior
leaflets are usually predominantly affected, whereas the
posterior leaflet may exhibit relatively preserved mobility.
Severe tricuspid regurgitation results in advanced stages
of tricuspid valve disease as the leaflets become fixed
in a semi-open position. There is also some degree of
concomitant stenosis due to a fixed orifice. Color flow
Doppler assessment of the hepatic veins may show
systolic flow reversal, consistent with severe tricuspid
regurgitation. On continuous wave Doppler tracings,
severe tricuspid regurgitation is characterized by a daggershaped profile with an early peak velocity and a rapid
decline, indicating rapid pressure equalization between
the right-sided cardiac chambers. The peak regurgitant
velocity may be increased due to coexistent pulmonic
stenosis. The presence of a prolonged pressure half-time
of the tricuspid inflow indicates associated tricuspid valve
stenosis. Increases in tricuspid inflow velocities and the
mean gradient across the tricuspid valve result from a
combination of valvular stenosis and increased blood flow
through the valve owing to the regurgitant volume.127
The pulmonary valve cusps appear thickened with
retraction and reduced mobility. The cusps may be difficult
to visualize if they are severely retracted. Constriction of
the pulmonary annulus as a result of plaque deposition
may also be observed. Doppler echocardiographic
assessment of the pulmonary valve is particularly helpful
because demonstration of the anatomical changes may be
challenging. Increased systolic velocities on continuous
wave Doppler examination are consistent with pulmonary
stenosis, whereas a dense regurgitant spectral profile with
a short deceleration time is typical for severe regurgitation.
1409
1410
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 66.40A to C: Carcinoid disease. Two-dimensional transthoracic echocardiography. (A) Apical four-chamber view shows
systolic noncoaptation of thickened anterior (1) and septal
(2) leaflets of tricuspid valve (TV); (B) Color Doppler examination
shows severe tricuspid regurgitation (arrowhead) resulting from
systolic noncoaptation of TV leaflets. Tricuspid regurgitation (TR)
jet practically fills the right atrium (RA); (C) Aortic (Ao) short-axis
view shows severe pulmonary regurgitation (PR) with the PR jet
extending all the way to the TV level. (LA: Left atrium; LV: Left
ventricle; PA: Pulmonary artery; RV: Right ventricle) (Movie clips
66.40A to C).
Source: Reproduced with permission from Ref. 128. 66.40A
to C).
Figs 66.41C to H
1411
1412
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
K
Figs 66.41A to K: Carcinoid disease. Live/real time three-dimensional transthoracic echocardiography. (A) 1, 2, and 3 represent
thickened anterior, septal, and inferior (posterior) leaflets of the tricuspid valve (TV), respectively, showing a very large area of
noncoaptation in systole (Movie clip 66.41A, part 1). Movie clip 66.41A part 2 shows QLAB images. Movie clip 66.41A part 3 shows color
Doppler assessment of TV regurgitation (TR). The vena contracta (VC; arrowhead) is very large, measuring 2.51 cm2, consistent with
torrential TR; (B) 1, 2, and 3 represent thickened anterior (left anterior), left (posterior), and right (right anterior) leaflets of the pulmonary
valve (PV), respectively, (Movie clip 66.41B part 1). In Movie clip 66.41B part 2 the arrowhead points to noncoaptation of the PV leaflets
in diastole. Movie clip 66.41B part 3 shows color Doppler assessment of pulmonary regurgitation (PR). The VC (arrowhead) is large,
measuring 0.7 cm2, consistent with severe PR; (C to E) Arrowhead shows a prominent, localized echogenic carcinoid deposit involving
the interatrial septum (IAS); (F) The arrowhead demonstrates a carcinoid deposit lining the wall of the inferior vena cava (IVC); (G and H)
QLAB. Upper arrowheads point to the carcinoid deposit involving the right atrium (RA) superior wall. Lower arrowhead views the same
plaque en-face. It measured 2.02 0.85 cm, area = 1.36 cm2; (I to K) Subcostal examination; (I) A large carcinoid metastasis, bounded
by dots, is noted in the liver (L). QLAB sections taken at two different levels show the extent of hemorrhage/necrosis (arrowheads) in
the lesion; (J) Another liver metastasis (M, dots) is seen encroaching the RA and IVC; (K) Examination of another patient with known
simple L cysts. Arrowheads point to some of the cysts, which are generally completely echolucent with thin, well-defined borders. These
features distinguish them from a carcinoid lesion, where the echolucencies do not involve the whole cyst and the borders are less well
defined and are thickened. (Ao: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral valve; PA: Pulmonary artery; RV: Right ventricle)
(Movie clips 66.41A parts 1 to 3, 66.41B parts 1 to 3, 66.41C to F, 66.41I parts 1 and 2, 66.41J and 66.41K).
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1. Richardson P, McKenna W, Bristow M, et al. Report of
the 1995 World Health Organization/International Society
and Federation of Cardiology Task Force on the Definition
and Classification of cardiomyopathies. Circulation.
1996;93(5):8412.
2. Maron BJ, Towbin JA, Thiene G, et al.; American Heart
Association; Council on Clinical Cardiology, Heart
Failure and Transplantation Committee; Quality of Care
and Outcomes Research and Functional Genomics and
Translational Biology Interdisciplinary Working Groups;
Council on Epidemiology and Prevention. Contemporary
definitions and classification of the cardiomyopathies:
an American Heart Association Scientific Statement
from the Council on Clinical Cardiology, Heart Failure
and Transplantation Committee; Quality of Care and
Outcomes Research and Functional Genomics and
Translational Biology Interdisciplinary Working Groups;
and Council on Epidemiology and Prevention. Circulation.
2006;113(14):180716.
3. Elliott P, Andersson B, Arbustini E, et al. Classification of the
cardiomyopathies: a position statement from the European
Society of Cardiology Working Group on Myocardial and
Pericardial Diseases. Eur Heart J. 2008;29(2):2706.
4. Lang RM, Bierig M, Devereux RB, et al. Chamber
Quantification Writing Group; American Society
of Echocardiographys Guidelines and Standards
Committee; European Association of Echocardiography.
Recommendations for chamber quantification: a report
from the American Society of Echocardiographys
Guidelines and Standards Committee and the Chamber
Quantification Writing Group, developed in conjunction
with the European Association of Echocardiography, a
branch of the European Society of Cardiology. J Am Soc
Echocardiogr. 2005;18(12):144063.
5. Kashani A, Barold SS. Significance of QRS complex
duration in patients with heart failure. J Am Coll Cardiol.
2005;46(12):218392.
6. Adabag S, Roukoz H, Anand IS, et al. Cardiac
resynchronization therapy in patients with minimal heart
failure: a systematic review and meta-analysis. J Am Coll
Cardiol. 2011;58(9):93541.
1413
1414
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
1415
1416
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
85. Ogbogu PU, Bochner BS, Butterfield JH, et al. Hypereosinophilic syndrome: a multicenter, retrospective
analysis of clinical characteristics and response to therapy.
J Allergy Clin Immunol. 2009;124(6):131925.e3.
86. Fauci AS, Harley JB, Roberts WC, et al. NIH conference.
The idiopathic hypereosinophilic syndrome. Clinical,
pathophysiologic, and therapeutic considerations. Ann
Intern Med. 1982; 97(1):7892.
87. Weller PF, Bubley GJ. The idiopathic hypereosinophilic
syndrome. Blood. 1994;83(10):275979.
88. Loeffler W. Endocarditis parietalis fibroplastica mit
Bluteosinophilic. Schweiz Me Wochenschr. 1936;66:817.
89. Tanino M, Kitamura K, Ohta G, et al. Hypereosinophilic
syndrome with extensive myocardial involvement and
mitral valve thrombus instead of mural thrombi. Acta
Pathol Jpn. 1983;33(6):123342.
90. Ommen SR, Seward JB, Tajik AJ. Clinical and echocardiographic features of hypereosinophilic syndromes.
Am J Cardiol. 2000;86(1):11013.
91. Feigenbaum H, Armstrong WF, Ryan T. Feigenbaums
Echocardiography. 6th ed. Philadelphia: Lippincott,
Williams & Wilkins; 2005:752.
92. Shah R, Ananthasubramaniam K. Evaluation of cardiac involvement in hypereosinophilic syndrome:
complementary roles of transthoracic, transesophageal,
and contrast echocardiography. Echocardiography. 2006;
23(8):68991.
93. Yacoub S, Kotit S, Mocumbi AO, et al. Neglected diseases
in cardiology: a call for urgent action. Nat Clin Pract
Cardiovasc Med. 2008;5(4):1767.
94. Rashwan MA, Ayman M, Ashour S, et al. Endomyocardial
fibrosis in Egypt: an illustrated review. Br Heart J.
1995;73(3):2849.
95. Mocumbi AO, Ferreira MB, Sidi D, et al. A population study
of endomyocardial fibrosis in a rural area of Mozambique.
N Engl J Med. 2008;359(1):439.
96. Acquatella H, Schiller NB. Echocardiographic recognition
of Chagas disease and endomyocardial fibrosis. J Am Soc
Echocardiogr. 1988;1(1):608.
97. Acquatella H, Schiller NB, Puigb JJ, et al. Value of twodimensional echocardiography in endomyocardial disease
with and without eosinophilia. A clinical and pathologic
study. Circulation. 1983;67(6):121926.
98. Vignaux O. Cardiac sarcoidosis: spectrum of MRI features.
AJR Am J Roentgenol. 2005;184(1):24954.
99. Uemura A, Morimoto S, Kato Y, et al. Relationship
between basal thinning of the interventricular septum and
atrioventricular block in patients with cardiac sarcoidosis.
Sarcoidosis Vasc Diffuse Lung Dis. 2005;22(1):635.
100. Matsumori A, Hara M, Nagai S, et al. Hypertrophic
cardiomyopathy as a manifestation of cardiac sarcoidosis.
Jpn Circ J. 2000;64(9):67983.
101. Fahy GJ, Marwick T, McCreery CJ, et al. Doppler
echocardiographic detection of left ventricular diastolic
dysfunction in patients with pulmonary sarcoidosis. Chest.
1996;109(1):626.
1417
CHAPTER 67
Echocardiographic Differentiation
of Ischemic and Nonischemic
Cardiomyopathy: Comparison with
Other Noninvasive Modalities
Sula Mazimba, Arshad Kamel, Navin C Nanda, Maximiliano German Amado Escanuela, Kunal Bhagatwala, Nidhi M Karia
Snapshot
Nonischemic Cardiomyopathy
M-Mode Echocardiography
Two-Dimensional/Three-Dimensional/Doppler
Echocardiography
INTRODUCTION
The left ventricle (LV) may be enlarged and show
dysfunction in both ischemic heart disease and dilated
cardiomyopathy. It is therefore important to distinguish
ischemic cardiomyopathy (ICM) from nonischemic dilated
cardiomyopathy (NICM) as management may be different.
The distinction between the two types of conditions may
have therapeutic and prognostic implications. Patients
with ICM may benefit from a revascularization treatment
strategy.13 Furthermore, patients with ICM have worse
prognosis than NICM patients.4 In clinical practice,
distinguishing between the two types of conditions can
be very challenging. In some situations, a diagnosis
can be inferred from history and physical examination
(e.g. postpartum or chemotherapy-induced NICM). In
general, distinction of the two cardiomyopathies lies in
1419
Fig. 67.2: Nonischemic cardiomyopathy. M-mode echocardiography shows a large mitralseptal separation, E-point septal
separation (EPSS) of 45 mm is noted on the M-mode. (AO: Aorta;
LA: Left atrium; LV: Left ventricle).
ECHOCARDIOGRAPHIC ASSESSMENT
OF ISCHEMIC AND NONISCHEMIC
CARDIOMYOPATHY
M-MODE ECHOCARDIOGRAPHY
The sphericity index (SI) is a surrogate marker of LV
remodeling. It is the ratio of left ventricular long-axis
internal dimension to the LV diameter at end-systole.30,31
A SI of <1.5 underscores a very severely dilated LV cavity.
Another M-mode parameter, which is related to LV cavity
dilatation and reduced LV systolic excursion, is the E-point
septal separation (EPSS). This is measured as the distance
from the tip of the open anterior mitral leaflet in diastole
1420
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 67.3A to F
1421
TWO-DIMENSIONAL/THREEDIMENSIONAL/DOPPLER
ECHOCARDIOGRAPHY
1422
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Two-dimensional Echo/Doppler-Derived
Left Ventricular Diastolic Dysfunction
Diastolic function assessment in NICM and ICM provides
useful information regarding the severity and overall
prognosis. Patients with restrictive diastolic physiology
on two-dimensional transthoracic echocardio
graphy
48,49
(2D TTE) have increased adverse outcomes.
A short
deceleration E time is associated with severe symptoms
and is also a powerful and independent prognostic
indicator of poor outcomes in patients with NICM.22,49
51
Grade II diastolic dysfunction has also been shown
to predict adverse outcomes and increased hospital
re-admissions in patients with NICM.52
Right Ventricle
Right ventricular (RV) dysfunction is a powerful predictor
for exercise capacity as well as mortality in patients with
NICM.5355 Assessment of RV function is often challenging
because of the complexity of the 3D structure that is
less amenable to geometric assumptions like the LV. In
addition, the RV is in a substernal position, which renders
imaging more challenging. The numerous trabeculations
also renders volumetric assessment of the RV function
challenging.56,57 3D TTE, however, compares favorably
with CMR in the assessment of RV function.58 A 2D TTE
parameter that has been validated in the assessment of RV
function is the tricuspid annular plane systolic excursion
(TAPSE).59,60 Right ventricular function is measured by the
degree of excursion of the tricuspid annulus from the base
to the apex. A TAPSE of <15 mm is associated with poor
RV systolic function. Decreased RV systolic function as
estimated by TAPSE is associated with increased mortality
in patients admitted for heart failure. Among patients with
LV systolic dysfunction, the involvement of RV dilatation is
often suggestive of a NICM. The RV is often spared in ICM
unless there is accompanying right ventricular infarction
in which case the right ventricle will be affected. RV
involvement portends worse adverse outcomes in both
NICM and ICM.55,6163
Left Atrium
Left atrial (LA) size has been shown to have prognostic
value in patients with NICM. LA volume is determined by
the degree of LV dilatation, diastolic dysfunction as well
as the severity of mitral regurgitation (MR).64,65 LA size
correlates well with the severity of diastolic dysfunction,
which in turn has prognostic information for patients
with both ICM and NICM.6672 In patients with NICM with
functional MR, LA size may reflect the duration of the MR.73
The LA size was a more powerful predictive variable than
the severity of MR jet in determining adverse outcomes
in NICM patients.65 The current American Society of
Echocardiography guidelines recommends obtaining
LA volumes rather than linear dimensions, which do
not take into account the asymmetric remodeling of
the chamber.21,74,75 The upper limit of normal LA-ESVI is
28 mL/m2.21
Tenting Area
This is the area enclosed by the annular plane and the two
mitral leaflets (Fig. 67.5). It has been shown to accurately
reflect the degree of functional mitral regurgitation
(FMR). It is also an independent predictor of mortality
and hospitalization in patients with NICM.95 Patients
with a tenting area > 3.4 cm2 had higher brain naturetic
peptide (BNP) levels, worse functional status, more
hospitalizations, and higher death rates.
1423
Fig. 67.5: The shaded region illustrates the tenting area of the
mitral valve. (H: Tenting height; LA: Left atrium; LV: Left ventricle;
MV: Mitral valve).
Fig. 67.4: Transesophageal apical four-chamber view showing
severe mitral regurgitation by color Doppler flow imaging
(arrow). Arrowhead shows a kink in the middle of the anterior leaflet
mimicking a seagull. This seagull sign results from tethering
produced by a strut chord and is considered indicative of ischemic origin of cardiomyopathy. (LA: Left atrium; LV: Left ventricle;
RV: Right ventricle) (Movie clip 67.4).
1424
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Stress Echocardiography
Stress echocardiography has emerged as a useful tool
for the evaluation of selected patients with suspected or
known ischemic heart disease.106 The high specificity of
stress echocardiography compared to other modalities
contributes to its utility as a cost-effective diagnostic tool
for CAD. The sensitivity and specificity of new WMAs
induced by dobutamine for detection of CAD is 89% and
85%, respectively. The sensitivity in those with multivessel
or left main disease is 100% compared to 81% in those
with a single vessel disease.107 Dobutamine stress echo
can help differentiate between ICM and NICM. In the
absence of CAD, the normal response to dobutamine
stress test is augmentation of systolic function and
contractility with increasing doses of dobutamine. In the
presence of significant disease, an initial augmentation
of LVEF and contractility is followed by a decline in
LVEF and/or contractility or emergence of new WMA
at higher doses of dobutamine.106,108 The segmental wall
motions are graded by dividing the heart into 16 segments
(17 segments if echo is being compared with another
imaging modality such as nuclear perfusion) and each
segment is then graded on a scale of 14 on the basis of
wall motion (1 = normal, 2 = hypokinesis, 3 = akinesis,
4 = dyskinesis).109 A cumulative score is obtained at
Coronary Echocardiography
2D TTE has been studied as a means of detecting CAD
and by extension, potentially differentiating ICM from
NICM.110 2D TTE was able to detect proximal CAD in
93% of the study patients. Interestingly, visualization of
the coronaries in dilated LV was much easier in patients
with NICM than ICM. This is primarily because NICM
patients generally have much larger chamber sizes than
ICM patients and, therefore, the curvature of the coronary
arteries is less steep and easily visualized. Although the
success rate of visualizing CAD lesions using 2D TTE was
high in this study, other researchers have recorded much
lower success rates.111113
ECHOCARDIOGRAPHIC DISTINCTION
BETWEEN ISCHEMIC CARDIOMYOPATHY AND NONISCHEMIC DILATED
CARDIOMYOPATHY
There are several features on 2D TTE that help distinguish
ICM from dilated NICM (Table 67.1). Traditionally, WMAs
have been used to discriminate between NICM and ICM.
In ICM, the WMA tend to be regional and correspond to
specific coronary artery distribution.119,120 The presence
of WMA has more diagnostic value in normal sized
ventricles.121125 Medina et al.121 studied 60 patients with
dilated LV and LV dysfunction using 2D TTE for the
detection of regional WMA so as to differentiate between
ICM and NICM. They reported a sensitivity, specificity, and
predictive accuracy of 83%, 57%, and 77%, respectively, in
differentiating ICM from NICM based on the presence of
WMA. In patients with normal LV size but LV dysfunction,
the sensitivity, specificity, and predictive accuracy
were found to be 95%, 100%, and 95%, respectively, in
detecting ICM.120 Besides WMA, identification of regional
thinning of myocardial wall (< 6 mm), or aneurysmal
myocardial segment corresponding to coronary blood
flow area increases the likelihood of the diagnosis of ICM.
Aneurysms and scar on the LV surface were found in fewer
than 15% of NICM on necropsies conducted by Roberts
et al.126 Chen et al. used semiquantitative echocardiographic
segmental wall motion scoring to predict CAD. Myocardial
wall motion was scored according to the scoring described
by Heger et al.127 (i.e. hyperkinesia = 1, normal = 0, hypoki
nesia = 1, akinesia = 2, and dyskinesis = 3). Patients with a
LVEF < 50% had a mean score of 6.9, while those with LVEF
above 50% had a mean score of 1.1.122
The above criteria are, however, not entirely exhaustive
in defining ICM because WMA may also be seen in up to
two-thirds of patients with NICM.107,128130 These WMA in
patients with NICM have been attributed to abnormal
microcirculatory perfusion despite normal epicardial
blood vessels.131,132 Wallis et al. showed that up to 64% of
patient with NICM had WMAs especially when conduction
abnormalities were present (i.e. left bundle branch block
[LBBB]). When they excluded patients with LBBB, WMA
were found in 59% of the study population with NICM.128
In the same study, Wallis et al. found that WMA were more
commonly associated with older age. The younger patients
with NICM had more diffuse involvement, were more
symptomatic, and generally had a poor prognosis. Because
resting WMA is not sensitive at discriminating CAD, the
1425
OTHER NONINVASIVE
IMAGING MODALITIES
Single-Photon Emission
Computed Tomography
Myocardial perfusion imaging (MPI) using singlephoton emission computed tomography (SPECT) is
a well-validated, noninvasive imaging modality used
in the diagnosis, treatment and prognostication of
CAD.136139 SPECT MPI has high sensitivity and specificity
that approaches 90% in detecting significant CAD (with
at least 50% luminal diameter stenosis).140142 In broad
terms, SPECT MPI classifies patients in three categories
normal study (normal perfusion defects at stress and
rest), reversible ischemia (perfusion defects with stress
and normal perfusion at rest), and a fixed scar (perfusion
abnormality at rest and stress). It is known that on SPECT
MPI, NICM is characterized by homogenous tracer
uptake, whereas ICM has extensive perfusion defects
that are often regional.138,143145 Patients with reversible
perfusion defects usually benefit from revascularization
treatment strategies. The limitation of SPECT with
thallium is that patients with NICM can still present
with perfusion defects even in the absence of significant
CAD.146 The combination of perfusion abnormalities that
occur in myocardial territories consistent with areas of
WMA overcomes the problem of WMA alone in NICM
undergoing thallium imaging alone.147 Large perfusion
defects, however, are more predictive of significant ICM
1426
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Table 67.1: Comparison of Noninvasive Findings in Ischemic Cardiomyopathy and Nonischemic Dilated Cardiomyopathy
Modality
Clinical evaluation
Older patients
May be younger
H
istory of ischemic heart disease such as angina
and myocardial infarction
S
egmental WMA/thinning (<6 mm in end diastole)
in coronary artery distribution. Sensitivity 65% to
81%, and specificity 56% to 99%
E
ndocardial brightening/scarring more common
due to infarction
Less common
B
iphasic response with dobutamine stress test
indicative of hibernating viable myocardium is
more common
Less common
P
erfusion defects with contrast echo more
common
Less common
M
ay show prominent plaques in larger vessels
such as ascending aorta, arch, and abdominal
aorta.
May be normal
A
symmetric closure of mitral valve leaflets with
eccentric regurgitation jet(s) more common.
Seagull sign (Fig. 67.4) may be present
Symmetric closure with central jets more common. Seagull sign absent
R
ight ventricle usually not enlarged and function
normal except when RCA is stenotic/occluded or
RV infarction present
Single-photon
emission computed
tomography (SPECT)
P
erfusion defects in coronary artery distribution.
More accurate if combined with WMA
Normal sized RV with good function more
common
Coronary computed
tomographic angiogram (CCTA)
C
oronary artery calcification more common.Coronary stenosis, both obstructive and
nonobstructive may be identified
Less common
Cardiac magnetic
resonance (CMR)
I ncreased gadolinium uptake or enhancement indicating scar formation in the previously infarcted
areas more common
Regional WMA/thinning and perfusion defects
similar to echocardiography above
Less common
Less common
Echocardiography
Positron emission
tomography (PET)
(2D TTE: Two-dimensional transthoracic echocardiography; ICM: Ischemic cardiomyopathy; LV: Left ventricle; NICM: Nonischemic
cardiomyopathy; RV: Right ventricle; WMAs: Wall motion abnormalities).
Coronary Computed
Tomographic Angiogram
Coronary computed tomographic angiogram (CCTA)
has emerged to be an important noninvasive diagnostic
modality in the evaluation of CAD.150152 The diagnostic
utility of CCTA is in patients with low to intermediate risk
for CAD. There are several published studies that have
looked at the role of CCTA in differentiating between
NICM and ICM.153157 Two primary avenues by which CCTA
stratifies patients for the underlying CAD is the calculation
of calcium score and by anatomical definition of coronary
arteries for presence of a plaque or stenosis. Calcium score
is calculated using electron-beam computed tomography
(EBCT) and multislice computed tomography (MSCT). A
high burden of coronary calcium (calcium score of > 80)
assessed by EBCT was associated with a 99% sensitivity
and an 83% specificity in accurately identifying patients
with ICM.154 Presence of calcium is a known marker
for atherosclerosis. In another imaging modality using
ultrasound of the carotids, carotid calcification in patients
with dilated cardiomyopathy had a sensitivity of 96% and
specificity of 89% of accurately identifying ICM.158 CCTA
is more sensitive in identifying patients with ICM than
2D TTE (sensitivity of 68% and specificity of 73%).159,160
However, the sensitivity of CCTA to accurately diagnose
ICM decreases in patients with a high calcium burden
(to 73%).156
1427
1428
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
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50.
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52.
53.
54.
55.
56.
57.
58.
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
74. Maddukuri PV, Vieira ML, DeCastro S, et al. What is the best
approach for the assessment of left atrial size? Comparison
of various unidimensional and two-dimensional parameters
with three-dimensional echocardiographically determined
left atrial volume. J Am Soc Echocardiogr. 2006;19(8):
102632.
75. Lester SJ, Ryan EW, Schiller NB, et al. Best method in
clinical practice and in research studies to determine left
atrial size. Am J Cardiol. 1999;84(7):82932.
76. Tahta SA, Oury JH, Maxwell JM, et al. Outcome after mitral
valve repair for functional ischemic mitral regurgitation.
J Heart Valve Dis. 2002;11(1):1118; discussion 1819.
77. Barzilai B, Davis VG, Stone PH, et al. Prognostic significance
of mitral regurgitation in acute myocardial infarction. The
MILIS Study Group. Am J Cardiol. 1990;65(18):116975.
78. Tomita T, Nakatani S, Eishi K, et al. Effectiveness of surgical
repair of mitral regurgitation concomitant with dilated
cardiomyopathy. J Cardiol. 1998;32(6):3916.
79. Blondheim DS, Jacobs LE, Kotler MN, et al. Dilated cardio
myopathy with mitral regurgitation: decreased survival
despite a low frequency of left ventricular thrombus. Am
Heart J. 1991;122(3 Pt 1):76371.
80. Borger MA, Alam A, Murphy PM, et al. Chronic ischemic
mitral regurgitation: repair, replace or rethink? Ann Thorac
Surg. 2006;81(3):115361.
81. Boltwood CM, Tei C, Wong M, et al. Quantitative echocar
diography of the mitral complex in dilated cardiomyopathy:
the mechanism of functional mitral regurgitation. Circu
lation. 1983;68(3):498508.
82. Trichon BH, OConnor CM. Secondary mitral and tricuspid
regurgitation accompanying left ventricular systolic dysfun
ction: is it important, and how is it treated? Am Heart J.
2002;144(3):3736.
83. Perloff JK, Roberts WC. The mitral apparatus. Func
tional anatomy of mitral regurgitation. Circulation. 1972;
46(2):22739.
84. Komeda M, Glasson JR, Bolger AF, et al. Geometric deter
minants of ischemic mitral regurgitation. Circulation.
1997;96(9 Suppl):II-12833.
85. Matsuzaki M, Yonezawa F, Toma Y, et al. Experimental
mitral regurgitation in ischemia-induced papillary muscle
dysfunction. J Cardiol Suppl. 1988;18:1216, discussion
127.
86. Kaul S, Spotnitz WD, Glasheen WP. et al. Mechanism of
ischemic mitral regurgitation. An experimental evaluation.
Circulation. 1991;84(5):216780.
87. Kono T, Sabbah HN, Rosman H, et al. Left ventricular shape
is the primary determinant of functional mitral regurgitation
in heart failure. J Am Coll Cardiol. 1992;20(7):15948.
88. Otsuji Y, Kumanohoso T, Yoshifuku S, et al. Isolated annular
dilation does not usually cause important functional mitral
regurgitation: comparison between patients with lone
atrial fibrillation and those with idiopathic or ischemic
cardiomyopathy. J Am Coll Cardiol. 2002;39(10):16516.
89. He S, Fontaine AA, Schwammenthal E, et al. Integrated
mechanism for functional mitral regurgitation: leaflet
restriction versus coapting force: in vitro studies. Circu
lation. 1997;96(6):18261834.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
CHAPTER 68
Pericardial Disease
Trevor Jenkins, Brian D Hoit
Snapshot
Acute Pericardi s
Pericardial Eusion
Pericardial Tamponade
INTRODUCTION
Pericardial heart disease represents a spectrum of
conditions with significant mortality and morbidity
commonly encountered in cardiovascular medicine.
Normal pericardial anatomy was first described in
antiquity, while descriptions of pericardial pathology
appeared during the 17th to 18th centuries.1 Ultrasound
visualization of the pericardium has advanced rapidly
during recent decades and has become an essential
tool for diagnosis and management of pericardial heart
disease. The value of echocardiography in this regard is
the ability to display both structure and physiology at
high spatial and temporal resolution. Efforts to visualize
pericardial disease originate with the early days of cardiac
ultrasound. Inge Edler first published images of an
anterior pericardial effusion in 1961.2 Harvey Feigenbaum
published several early manuscripts documenting the
power of echocardiography to display pericardial effusions
and published the relation between the swinging heart
in cardiac tamponade by M-mode with electrical alternans
by electrocardiography (ECG).3 Such early observations
helped expand the scope of echocardiography beyond
detection of mitral stenosis.4
Constric ve Pericardi s
Eusive-Constric ve Pericardi s
Congenital Anomalies
1436
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Mechanical
Effects on individual chambers:
ACUTE PERICARDITIS
Acute pericarditis may be isolated or present as a
manifestation of a systemic process. Although the etiology
is highly variable, most cases of acute pericarditis are
idiopathic or viral. Inflammation of the pericardium
is usually silent echocardiographically, as echogenic
brightness of the pericardium lacks sufficient diagnostic
sensitivity and specificity.
Echocardiography is recommended as the initial
noninvasive imaging test for acute pericarditis, because
it accurately detects pericardial effusion and tamponade,
and ventricular dysfunction due to myopericarditis.9
Echocardiography estimates the volume of pericardial
fluid, identifies cardiac tamponade, suggests the basis of
pericarditis, and documents associated acute myocarditis.
In addition, the presence of adhesions, fibrous strands,
hemorrhage, and loculations may aid in the diagnosis of
morbid conditions such as purulent bacterial pericarditis
that may require pericardiocentesis. Although patients
with purely fibrinous acute pericarditis have a normal
echocardiogram, the presence of a pericardial effusion is
consistent with acute pericarditis and is one of the criteria
for its diagnosis. A transthoracic echocardiogram (TTE) is
particularly critical in the setting of high-risk features of
acute pericarditis associated with worse outcome including
fever > 38C, subacute onset, an immunosupressed state,
trauma, or evidence of hemodynamic compromise.10
The use of echocardiography for the evaluation of all
patients with suspected pericardial disease was given
a Class I recommendation by a 2003 task force of the
American College of Cardiology (ACC), the American
Heart Association (AHA), and the American Society of
Echocardiography (ASE).11 Additional imaging modalities
may be necessary if the TTE is negative or inconclusive in
a patient with complex or atypical clinical presentations.
PERICARDIAL EFFUSION
Accumulation of transudative or exudative fluid in excess
of 50 mL is abnormal and may be seen with pericarditis
Idiopathic:
Infectious (viral, bacterial, mycobacterial, fungal, or AIDS/HIV)
Autoimmune (systemic lupus erythematosus, rheumatoid
arthritis, systemic sclerosis, or ankylosing spondylitis)
Neoplastic [primary (mesothelioma), secondary (breast,
lung, melanoma, lymphoma)]
Radiotherapy:
Nephrogenic (uremic, dialytic)
Cardiac injury (surgery, interventional, trauma)
[acute
or
chronic
Myocardial infarction
syndrome)]
1437
(Dresslers
1438
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 68.1A to D: Two-dimensional (2D) echocardiography of pericardial effusions of varying size. Anterior (*) and posterior (arrow) are
seen as echo-free spaces of increasing size including small, posterior (A), moderate circumferential (B), and large circumferential (C).
Figure D shows a large pleural effusion (curved arrow) posterior to a small pericardial effusion (arrow) with the pericardium visualized as
an echogenic linearity between both. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
PERICARDIAL TAMPONADE
Cardiac tamponade is a life-threatening condition
caused by fluid accumulation in the pericardial sac and
is characterized by elevation and equalization of cardiac
diastolic and pericardial pressures, a reduced cardiac
output, and an exaggerated inspiratory decrease in arterial
systolic pressure (>10 mm Hg) referred to as pulsus
paradoxus. Cardiac tamponade is poorly related to the size
of the effusion, as it is the rapidity of fluid accumulation in
the pericardial space and the eclipse of pericardial reserve
1439
Figs 68.2A to D: M-mode echocardiography of pericardial effusions of varying size. Anterior (*) and posterior (arrow) are seen as
echo-free spaces of increasing size including small, posterior (A), moderate circumferential (B), and large circumferential (C). Concurrent pleural (curved arrow) and pericardial effusion are shown in Figure D. Note that parietal pericardium displays relatively flat motion
throughout the cardiac cycle best visualized in Figure C. (LV: Left ventricle; RV: Right ventricle).
Figs 68.3A and B: Exudative pericardial effusions as seen by two-dimensional (2D) echocardiography apical views. Frond-like (A) and
band-like (B) adhesions are seen bridging a large pericardial effusion fluid, indicating an inflammatory component to the effusion. Note
the thickened pericardium in Figure A. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
1440
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
1441
Figs 68.5A and B: Pseudohypertrophy (A) is demonstrated in the setting of a large pericardial effusion with tamponade physiology with
normalization; (B) of left ventricular wall thickness after pericardiocentesis.
Fig. 68.6: Plethora (dilation) of the inferior vena cava (IVC) indicative of elevated central venous filling pressure in the setting of
tamponade. (L: Liver).
1442
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 68.7A and B: Two-dimensional (2D) echocardiograms in the apical four-chamber view demonstrating chamber collapse due to
tamponade. During late diastole, there is inversion of the right atrial lateral wall (A), and right ventricular free wall (B). (AO: Aorta; LV:
Left ventricle; RA: Right atrium; RV: Right ventricle).
90
65
Right atrium
68
66
Right ventricle
60
90
45
92
1443
Figs 68.8A and B: Pulsed wave Doppler in a patient with cardiac tamponade. Note the increased expiratory flow velocity of the mitral
valve (A) and increased inspiratory flow velocity of the tricuspid valve (B).
Echo-Guided Pericardiocentesis
Unless the situation is immediately life-threatening,
experienced staff should perform pericardiocentesis in a
facility equipped with monitoring to optimize the success
and safety of the procedure. Monitoring the cardiac rhythm
and systemic blood pressure is a minimum requirement.
The advantages of needle pericardiocentesis include
the ability to perform careful hemodynamic measurements
1444
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 68.10A and B: Two-dimensional (2D) echocardiography-assisted pericardiocentesis from the apical four-chamber view. Note the
appearance of agitated saline bubbles (Figure A arrow) after successful needle entry into the pericardial space. Catheter advancement
(Figure B arrow) is visualized as the pericardial drain is passed into the effusion. (LV: Left ventricle; RV: Right ventricle).
CONSTRICTIVE PERICARDITIS
Constrictive pericarditis (CP) is a condition in which
a thickened, scarred, inelastic, noncompliant, and
often calcified pericardium limits diastolic filling of the
ventricles. The etiologies of CP are wide-ranging and
include viral and idiopathic pericarditis, cardiac surgery
(the most common antecedent in developed countries),
tuberculosis (common in underdeveloped countries),
collagen vascular disease, trauma, and chest radiation.
CP may have a long latency period after the initial (and
sometimes unrecognized) pericardial injury as might
occur in post-irradiation and post-traumatic pericarditis,
becoming apparent only decades later. Although it is
commonly thought that a normal pericardial thickness
excludes the diagnosis of CP, 28% of 143 surgically
confirmed cases had normal pericardial thickness on CT
scan, and 18% had normal thickness on histopathological
examination.31
Classic chronic CP is encountered less frequently
than it was in the past, whereas subacute CP, occurring
weeks to months after the inciting injury, is becoming
1445
M-Mode:
Fig. 68.11: M-mode echocardiogram from a patient with constrictive pericarditis. Note the echo-bright thickened posterior
pericardium and flat posterior left ventricular (LV) wall during mid
to late diastole (arrow). An atrial systolic notch (*) is visualized
after atrial systole.
Doppler:
Restrictive diastolic filling pattern on mitral and tricuspid
inflow
E/A velocity ratio > 1.5 with shortened deceleration time
(< 150 ms)
Tricuspid inflow velocity increases on first beat after
inspiration
Tissue Doppler:
Annulus paradoxus
Annulus reversus
1446
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 68.12A and B: Two-dimensional (2D) echocardiography findings in a patient with constrictive pericarditis. Note the tubular geometry
of left ventricle in parasternal long-axis (A) and apical four-chamber (B) views. Figure A demonstrates DCruz sign, an abnormal contour
of the posterior left ventricle and posterior left atrial walls giving rise to an angle < 150. (LA: Left atrium; LV: Left ventricle; RA: Right
atrium; RV: Right ventricle).
1447
Figs 68.15A and B: Tissue Doppler velocities in a patient with constrictive pericarditis. There are normal lateral (A) and supranormal
septal (B) early diastolic tissue velocities. Note the annulus reversus sign with higher septal than lateral early diastolic velocity. This
sign is likely due to tethering of the lateral atrioventricular groove to the thickened pericardium.
1448
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 68.16A and B: Two-dimensional (2D) echocardiogram (A) and M-mode of effusive constrictive pericarditis from the parasternal
view. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle).
EFFUSIVE-CONSTRICTIVE
PERICARDITIS
EffusiveCP is an uncommon syndrome of pericardial
constraint with clinical and pathophysiological features
of both cardiac tamponade and chronic CP. Idiopathic
pericarditis, radiation, and neoplasia are the most
frequent antecedents. Unlike the predominant parietal
involvement in chronic CP, involvement of the visceral as
well as the parietal pericardium (epicarditis) is involved in
effusiveCP with inflammation, fibrotic thickening, and
variable degrees of myocardial adherence.48 In a series of
190 patients undergoing pericardiocentesis for cardiac
tamponade, the disorder was diagnosed in 15 (8%).49
The diagnosis was defined by a failure of the right atrial
pressure to fall by 50% or to a level below 10 mm Hg after
pericardiocentesis. In these patients, constriction was
clinically suspect in 7; symptoms were usually present
for less than 3 months, right-heart failure was evident in
all, evidence of acute pericarditis was noted in 7, pulsus
paradoxus was noted in 10, and pericardial calcification
was present in none.
Noninvasive imaging is not very useful in the diagnosis
of effusiveCP.50 The echo findings of effusiveCP depend
on the stage of the disease, although most often the
M-mode, 2D, and Doppler features are consistent with
a moderate or large pericardial effusion and cardiac
tamponade. The pericardial effusion may become
organized; echogenic and fibrinous strands may result in
regions of loculation (Figs 68.16A and B).
CONGENITAL ANOMALIES
Pericardial cysts are usually rare remnants of defective
embryological development of the pericardium. In
1449
Figs 68.17A and B: A pericardial metastasis near the right ventricle (A) is seen in patient with widely metastatic lymphoma in the
subcostal window. Definity contrast enhancement (B) opacifies the right ventricle (RV) blood pool. (L: Liver).
Figs 68.18A and B: A pericardial cyst (arrow) is seen adjacent to the right ventricle. Color Doppler flow (B) within the right atrium
demonstrates normal vena caval flow but a lack of flow within the pericardial cyst. (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).
1450
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
REFERENCES
1. Shabetai R. The Pericardium. 2nd ed. Norwell, MA: Kluwer
Academic; 2003: 129.
1451
CHAPTER 69
Three-Dimensional Echocardiographic
Assessment in Pericardial Disorders
O Julian Booker, Navin C Nanda
Snapshot
INTRODUCTION
The pericardium is a relatively avascular fibroserous sac.
It is created by invagination of the heart within the serous
pericardium and surrounded by the fibrous pericardium.
The serous pericardium is composed of a single layer
of mesothelial cells. The layer adherent to the fibrous
pericardium is termed the parietal layer. The layer deep to
the fibrous pericardium is called the visceral pericardium.
Those portions of the visceral pericardium that are
adherent to the myocardium are specifically called the
epicardium. The fibrous pericardium is much sturdier
than the serous pericardium but is still relatively thin
(< 2 mm) and surrounds the heart and part of the great
vessels.1 Between the serous layers, there is a potential
space that contains 15 to 50 mL of fluid that serves
as lubrication. Most of the fluid is located within the
interventricular and atrioventricular grooves with a
predilection for more dependent areas.
The pericardium has a unique interaction with heart.
The fibrous pericardium attaches to the diaphragm,
anterior mediastinum, and sternum. The pericardium
surrounds the proximal portions of the great vessels, which
serve as additional anchor points. These attachments serve
to fix the heart within the chest. The nature and position of
the fibrous pericardium also make it an important barrier
to infection. Pericardial constraint allows for greater
isovolumic pressures at any given volume for both the
left and the right ventricles. These benefits of constraint
are curbed by the absence of the pericardium.24 Further
possible roles of pericardial constraint include preventing
acute increases in chamber volume as a response to
elevations in filling pressures. It also helps modulate
the physical and hydrodynamic interaction between
the four cardiac chambers5,6 and creates ventricular
interdependence.7,8
There are anatomical and hemodynamic consequences
to pericardial disease. The cardiac chambers are physically
connected within a confined space within the pericardium.
There is also hydrodynamic interdependence related to
blood flow. Changes in the filling dynamics of one chamber
will impact the others. Tamponade and constriction are
primarily hemodynamic entities resulting from abnormal
pericardial constraint. Echocardiography, with its ability
to not only evaluate structure but also hemodynamic
assessment, is the cornerstone of the evaluation of
pericardial disorders. The standard evaluation involves
Doppler interrogation and two-dimensional (2D) imaging.
Over the past decade, more powerful computer processors have resulted in larger quantities of data that can
be processed quickly. Development of a full matrix array
has evolved echocardiography from an approximately
5 mm slice to a nearly full volumetric acquisition of the
heart and its immediately surrounding structures using a
gated, multiple-beat acquisition. Three-dimensional (3D)
technology provides the ability to manipulate and crop the
data set in an almost infinite number of orientations and
provides a more complete assessment of cardiac anatomy
including the pericardium. With high-resolution images,
the surfaces of the two layers can be viewed en face.9 This
approach allows for a more complete structural assessment
than two-dimensional transthoracic echocardiography
(2D TTE) alone.
Publications regarding three-dimensional transthoracic echocardiography (3D TTE) in the evaluation of pericardial disease have been limited. Three-dimensional echocardiographys more complete anatomical visualization
1453
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Contd...
Differentiation of pericardial effusion from pleural effusion and ascites
En-face viewing of the falciform ligament to better identify ascites
Pericardiocentesis
Better needle visualization with its pyramidal imaging plane
Pericardial cyst
Identification of loculations and trabeculations
*3D TTE, live/real time three-dimensional transthoracic echocardiography.
Source: Reproduced with permission from Sudhakar S, Nanda NC. Role of live/real time three-dimensional transthoracic echocardiography in pericardial disease. Echocardiography. 2012;29:98102.
Figs 69.1A to D: Live/real-time three-dimensional transthoracic echocardiography in pericardial effusion in a 64-year-old female with
renal failure. (A) The arrowhead points to fibrin deposition over the right ventricular pericardium giving a rugged appearance. Pericardial
effusion extends behind both atrial (see also Movie clip 69.1A). (B) Apical four-chamber view. Cropping from bottom displays a smooth
visceral pericardium over the basal left atrial (LA) wall (arrowhead; see also Movie clip 69.1B). (C) Subcostal examination. Arrowheads
show multiple fibrin deposits on the right atrial (RA) visceral pericardium resulting in a rugged appearance (see also Movie clip 69.1C).
(D) Arrowhead shows a flap-like fibrin mass (see Movie clip 69.1D parts 1 and 2). (IVC: Inferior vena cava; LV: Lleft ventricle; PE: Pericardial effusion; TV: Tricuspid valve).6
Source: Reproduced with permission from Ref. 10.
1455
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Fig. 69.3: Live/real time three-dimensional transthoracic echocardiography in an 84-year-old male with renal failure, and
pericardial and left pleural effusions. Cropping of the threedimensional data set using Qlab software analysis package
revealed a rugged visceral (1, 2, arrowhead) and parietal
(3) pericardium as well as a rugged visceral (4) and parietal
(5) pleura, from fibrin deposits (see also Movie clip 69.3A). Movie
clips 69.3B and C show similar findings using regular cropping in
the same patient. Movie clips 69.3D and E are from a different
patient with pleural effusion, shown for comparison. These show
cropping to more comprehensively assess the extent of pleural
effusion (PLE) and the collapsed lung (arrowhead) and their
relation to the heart (H). Movie clip 69.3F from another patient
with chronic renal failure shows the attachment of collapsed lung
lobes (horizontal arrowheads) to the hilum (vertical arrowheads)
and its relationship to the heart (H). This patient had previously
undergone pericardiectomy for constriction. Both these patients
were studied from the back in the sitting position. (DA: Descending
thoracic aorta; PP: Parietal pericardium; VPL: Visceral pleura;
VP: Visceral pericardium).
Source: Reproduced with permission from Ref. 10.
Fig. 69.4: Live/real time three-dimensional transthoracic echocardiography in a 62-year-old male with pericardial effusion
developing following mitral valve replacement. Arrowheads point
to fibrinous strands connecting the visceral and parietal portions of
the pericardium. The accompanying Movie clips 69.4A and B from
the same patient show both mobile and nonmobile fibrin strands. In
Movie clip 69.4A, artifacts appeared when the instrument gain was
decreased and disappeared with increase in gain, emphasizing
the importance of optimizing gain settings when assessing threedimensional images. Movie clip 69.4B shows cropping done using
the Qlab software analysis package. Abbreviations as in previous
figures.
Source: Reproduced with permission from Ref. 10.
1457
Fig. 69.5: Live/real time three-dimensional transthoracic echocardiography in a 50-year-old male status post cardiac transplantation and pericardial effusion compressing the right atrium.
The arrowhead points to the compressed right atrium (RA). Cropping reveals no significant echo reflectors (solid tissue) in the
effusion suggesting that it mainly comprises fluid or represents a
hematoma that is of uniform, homogenous consistency (see also
Movie clip 69.5). The patient improved after drainage of 1,000 cc
of yellowish fluid from the pericardial cavity using a pigtail catheter.
(Abbreviations as in previous figures).
Source: Reproduced with permission from Ref. 10.
Fig. 69.6: Live/real time three-dimensional transthoracic echocardiography in a 17-year-old male with a bullet injury and subsequent development of pericardial hematoma. The red dots outline
a loculated component of a very large pericardial hematoma (see
also Movie clip 69.6A). Movie clip 69.6B shows a huge pericardial
hematoma (arrowhead) with large multiple echolucencies consistent with fluid collections. These were not well seen on two-dimensional imaging. (Abbreviations as in previous figures).
Source: Reproduced with permission from Ref. 10.
1458
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 69.7A to D: Live/real time three-dimensional transthoracic echocardiography in a 26-year-old male with tuberculous pericardial
effusion. (A) Cropping of the apical four-chamber data set shows no inward motion of the proximal right ventricular free wall (arrowhead)
during systole, probably due to fibrinous adhesions. The distal wall contracts well; (B) Examination of visceral pericardium (VP) over
the ventricles demonstrates a mildly rugged appearance. Movie clips 69.7A and B Parts 1 to 3 show the cropping technique used to
demonstrate the visceral pericardium of both ventricles; (C) A large mass (arrowhead) is seen involving the visceral pericardium of the
left ventricle (LV). The etiology is not clear but it could possibly represent a tuberculous granuloma; (D) The arrowhead points to a large
highly echogenic mass involving the right ventricular visceral pericardium consistent with a calcified granuloma in another patient with
tuberculosis. Movie clip 69.7C from the same patient shows a granuloma (arrowhead) involving the parietal pericardium. Movie clips
69.7D and E are from a different patient with purulent pericardial effusion due to methicillin-resistant Staphylococcus aureus. Movie clip
69.7D shows markedly thickened and echogenic parietal (upper arrowhead) and visceral (lower arrowhead) pericardium. Movie clip
69.7E shows an abnormal loculated appearance of the visceral pericardium (arrowhead) when viewed en-face by cropping from the
apex. (Abbreviations as in previous figures).
Source: Reproduced with permission from Ref. 10.
1459
Figs 69.8A and B: Live/real time three-dimensional transthoracic echocardiography in a 66-year-old male with pericardial metastasis
from a malignant thymoma. (A) The arrowhead shows a huge pericardial mass (bounded by red dots) measuring 9.2 6.3 cm. Movie
clips 69.8A to C show the full extent of this huge mass with three-dimensional imaging. Cutting open the tumor in its mid portion (arrowhead in Movie clip 69.8B) revealed solid inhomogenous tissue. Arrowheads in Movie clip 69.8C show parietal involvement of the tumor
by multiple band-like extensions. The two-dimensional study in this patient (Movie clip 69.8D) shows a much smaller mass (arrowhead)
measuring 3.8 1.5 cm attached to the right ventricular outflow tract visceral pericardium; (B) Surgical specimen. (Other abbreviations
as in previous figures).
Source: Reproduced with permission from Ref. 10.
Figs 69.9A and B: Live/real time three-dimensional transthoracic echocardiography in a 68-year-old male with lung carcinoma. (A and B)
The arrowhead points to an irregular mass in the parietal pericardium (see also Movie clips 69.9A and B). The etiology is not clear,
but it could possibly represent a pericardial metastasis. Movie clip 69.9C shows another mass (arrowhead) located over the visceral
pericardium (VP) in this patient. This type of mass (arrowhead) was also seen by two-dimensional imaging (Movie clip 69.9D). However,
the other mass seen involving parietal pericardium (PP) was not detected by this modality. Movie clip 69.9E is from a different patient
with a poorly differentiated lung adenocarcinoma. Arrowhead points to a mass in the parietal pericardium consistent with metastasis.
This was not visualized on two-dimensional imaging. Examination of pericardial fluid in this patient showed the presence of malignant
cells. (Other abbreviations as in previous figures).
Source: Reproduced with permission from Ref. 10.
1460
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 69.10A and B: Live/real time three-dimensional transthoracic echocardiography in a 36-year-old male with a pericardial cyst. (A)
The arrowhead points to a pericardial cyst confirmed by a CT scan of the chest (see Movie clip 69.10A). (B) Cropping of the threedimensional data set demonstrates multiple band-like tissue (arrowhead) within the cyst (see Movie clip 69.10B). Movie clips 69.10C
and D represent two-dimensional images which do not show multiple bands criss-crossing the cyst (arrowhead). (L: Liver). (Other
abbreviations as in previous figures).
Source: Reproduced with permission from Ref. 10.
Figs 69.11A to C: Two-dimensional and live/real time threedimensional transthoracic echocardiography in a 53-yearold female with constrictive pericarditis. (A) Two-dimensional
study. Arrowheads in the parasternal long-axis view point to
an echogenic left ventricular posterior wall consistent with
calcification (see also Movie clip 69.11A); (B) Represents a threedimensional study using Qlab software analysis package. The
arrow demonstrates a highly echogenic posterior pericardium
consistent with calcification. When this was cropped transversely
using an oblique cropping plane, widespread involvement of the
left ventricular posterior wall with calcification (arrowhead) was
evident. Highly echogenic calcification is visualized anteriorly also.
Top and bottom arrowheads in the left upper quadrant in Movie clip
69.11B point to anterior and posterior calcification, respectively.
The patient underwent pericardiectomy. (AO: Aorta; MV: Mitral
valve). (Other abbreviations as in previous figures).
Source: Reproduced with permission from Ref. 10.
CONCLUSION
Pericardial diseases vary widely in their nature and
consequences. Although 2D TTE is the imaging modality of
choice to evaluate pericardial conditions, its planar nature
can lead to incomplete visualization of the pericardium.
3D TTE has shown benefit in both the diagnosis of
pericardial disease and guiding therapeutic intervention.
Including a 3D data set should become part of the routine
echocardiographic evaluation of pericardial disease where
the diagnosis is in question or if intervention is required.
REFERENCES
1. Choi HO, Song JM, Shim TS, et al. Prognostic value of initial
echocardiographic features in patients with tuberculosis
pericarditis. Korean Circ J. 2010;40(8):37786.
2. Shabetai R, Mangiardi L, Bhargava V, et al. The pericardium
and cardiac function. Prog Cardiovasc Dis. 1979;22:
10734.
3. Fowler NO, Gabel M, Holmes JC. Hemodynamic effects
of nitro-prusside and hydralazine in experimental cardiac
tamponade. Circulation. 1978;57:5637.
4. Glantz SA, Misbach GA, Moores WY, et al. The pericardium
substantially affects the left ventricular diastolic pressurevolume relationship in the dog. Circ Res. 1978;42:43341.
5. Maruyama Y, Ashikawa K, Isoyama S, et al. Mechanical
interactions between four heart chambers with and
without the pericardium in canine hearts. Circ Res. 1982;50:
86100.
1461
CHAPTER 70
Echocardiographic Assessment of
Cardiac Tumors and Masses
Leon Varjabedian, Jennifer K Lang, Abdallah Kamouh, Steven J Horn, Tuba Kemalolu z, Aylin Sungur, Kruti Jayesh
Mehta, Kunal Bhagatwala, Nidhi M Karia, Maximiliano German Amado Escauela, Robert P Gatewood Jr, Navin C Nanda
Snapshot
ECHOCARDIOGRAPHIC ASSESSMENT
OF CARDIAC TUMORS AND MASSES
Standard two-dimensional (2D) transthoracic echocardiography (TTE) with Doppler is the initial diagnostic
modality of choice in a patient with a cardiac tumor or
mass. Transesophageal echocardiography (TEE) is often
complementary to TTE in the full assessment of patients
with a known or suspected cardiac mass.
A complete echocardiographic evaluation of a cardiac
tumor or mass should include: (a) Characterization of
the shape, dimensions, and volume of the mass (e.g.
round vs lobulated, small vs large).1 The size of an
intracardiac mass has important clinical implications
in predicting embolic events, congestive heart failure,
and death, and as an efficacy assessment after treatment
(anticoagulation, antibiotics, and chemotherapy).2 Nanda
et al. reported that 2D measurements from a transthoracic
or a transesophageal study underestimate the true
maximum diameter of irregularly shaped structures. (b)
Identification of the location of the tumor and type of
attachment to the heart (e.g. pedunculated vs sessile).
(c) Description of the echogenicity (e.g. echolucent vs
echodense, homogenous vs heterogeneous, and presence of calcification). (d) assessment of mobility (e.g.
MICE
Extracardiac Masses
1463
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1465
Figs 70.2A and B: Transesophageal echocardiogram of a patient with a tricuspid valve myxoma. (LA: Left atrium; LV: Left ventricle;
RA: Right atrium; RV: Right ventricle) (Movie clip 70.2.).
Figs 70.3A to C
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Figs 70.3A to G: Live/real time three-dimensional (3D) transthoracic echocardiographic assessment of left atrial myxoma. (A
to C) Frontal plane sections taken at three different sequential
levels in the 3D data set demonstrate echolucencies (arrowhead)
within the tumor consistent with hemorrhage, which is largest in
the middle section (B); (D) Transverse plane sections of the tumor
viewed en face, also demonstrating hemorrhage (arrowhead);
(E) Frontal plane, transverse plane, and vertical plane sections of
the tumor showing extensive hemorrhage (arrowhead); (F) The
arrow points to the tumor stalk; (G) Resected specimen showing
a large hemorrhage. Arrows in the Movie clip 70.3 point to large
areas of hemorrhage that correspond with the surgical specimen.
(AO: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral
valve; RA: Right atrium; RV: Right ventricle) (Movie clip 70.3).
Source: Reproduced with permission from Mehmood F, Nanda
NC, Vengala S, et al. Live three-dimensional transthoracic echocardiographic assessment of left atrial tumors. Echocardiography.
2005;22(2):13743.
1467
Figs 70.4A and B: Live/real time three-dimensional transthoracic echocardiographic assessment of left atrial myxoma. (A) Arrowhead
points to a large echolucency consistent with a large hemorrhage. (B) Resected specimen showing a large hemorrhage. (LA: Left
atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Source: Reproduced with permission from Mehmood F, Nanda NC, Vengala S, et al. Live three-dimensional transthoracic echocardiographic assessment of left atrial tumors. Echocardiography. 2005;22(2):13743.
Figs 70.5A and B: Live/real time three-dimensional transthoracic echocardiographic assessment of left atrial myxoma. (A) Arrowhead
points to a large echolucency, which corresponds closely to the hemorrhage seen in the resected specimen (B). (LA: Left atrium; LV:
Left ventricle; MV: Mitral valve; RA: Right atrium; RV: Right ventricle).
Source: Reproduced with permission from Mehmood F, Nanda NC, Vengala S, et al. Live three-dimensional transthoracic echocardiographic assessment of left atrial tumors. Echocardiography. 2005;22(2):13743.
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Figs 70.6A to D: Two-dimensional (A) and live/real time three-dimensional (3D; B and C) transesophageal echocardiography.
(A) Arrow points to a myxoma in the left atrium with a broad attachment on the atrial septum. The dense areas represent calcification,
while the echolucent areas indicate the presence of hemorrhages. (B and C) The attachment of the tumor is much better delineated by
the 3D technique. (IAS: Interatrial septum; LA: Left atrium; RA: Right atrium); (D) Schematic diagram demonstrating that the maximum
dimension of an object (in this case, a cylinder) can be obtained only if the ultrasound beam cuts through its longest dimension (true
long axis) when using a multiplane probe. However, when the two-dimensional planes (dotted lines) are stacked together to obtain a 3D
image, the object (cylinder), including its long axis, can be viewed completely, even though it is not oriented parallel to the ultrasound
beam as it is rotated from 0 to 180 (Movie clips 70.6A to C).
Source: Reproduced with permission from Nanda NC et al. Incremental value of three-dimensional echocardiography over
transesophageal multiplane two-dimensional echocardiography in qualitative and quantitative assessment of cardiac masses and
defects. Echocardiography. 1995;12:61928.
1469
Figs 70.7A to F: Transesophageal echocardiogram of a patient with a large left atrial septal myxoma (A to F) causing significant mitral
regurgitation (E and F). (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle) (Movie clips 70.7A and B).
1470
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Figs 70.8A and B: Two-dimensional transesophageal echocardiography in a patient with a large biatrial myxoma presenting for
operation. (A) Arrowheads point to areas of calcification within the tumor. (B) Arrowheads point to areas of hemorrhage within the tumor.
(AS: Atrial septum; AV: Aortic valve; H: Hemorrhage; LA: Left atrium; LVO: Left ventricular outflow tract; M1: Myxoma in right atrium; M2:
Myxoma in left atrium; RA: Right atrium; RVO: Right ventricular outflow tract).
Source: Reproduced with permission from Srivastava R, Hsiung MC, Fadel A, Nanda NC. Transesophageal echocardiographic
demonstration of biatrial myxoma. Echocardiography. 2004;21(2):1878.
Figs 70.9A and B: (A) Parasternal long-axis and (B) apical four-chamber views demonstrating a large right atrial myxoma attached
to the atrial wall by a stalk prolapsing through the tricuspid valve into the right ventricle. (RA: Right atrium; RV: Right ventricle) (Movie
clip 70.9).
1471
C
Figs 70.11A to C
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1473
Figs 70.12A to D: Real time two- (A) and three-dimensional (B to D) transthoracic echocardiography in right ventricular myxoma. (A)
The arrowhead points to a large myxoma in the right ventricle (RV) visualized in the apical four-chamber view. Tumor attachment is not
visualized. (B to D) The arrowhead in B points to the large myxoma. The arrowheads in C show attachments of the myxoma to the RV
inferior wall; D shows one of the attachments (arrow) of the tumor to the tricuspid valve using the QLab on the system. (LA: Left atrium;
LV: Left ventricle; RA: Right atrium) (Movie clips 70.12 BD Parts 1 and 2).
Source: Reproduced with permission from Reddy VK, Faulkner M, Bandarupalli N, et al. Incremental value of live/real time three-dimensional
transthoracic echocardiography in the assessment of right ventricular masses. Echocardiography. 2009;26(5):598609.
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Papillary Fibroelastoma
Papillary fibroelastomas (PFEs) are the second most
common primary cardiac neoplasms, accounting for 5% of
all tumors of the heart52 and 7.9% of benign primary cardiac
tumors. However, they are the most common valvular
tumors.53,54 The true incidence may be underestimated55
because PFEs are often asymptomatic. It is frequently
observed between the fourth and the eighth decade of
life.56 The pathophysiology of this tumor origin is variable.
It can be seen in patients with long-standing heart disease
1475
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
B1
B2
Figs 70.17A and B: (A) Two-dimensional transthoracic echocardiography (parasternal long-axis view) of a patient with a papillary
fibroelastoma presenting as an 0.8 0.8 cm mobile echogenic
mass at the level of the sinotubular junction attached to the aortic surface of one of the aortic valve leaflets. Source: Dr Hashmat
Ashraf, Chief of Cardiac Surgery, Buffalo General Medical Center,
Buffalo, NY; (B) Two-dimensional transesophageal echocardiography showing a papillary fibroelastoma attached to the aortic valve
causing aortic insufficiency (AI) as shown by color Doppler (right
panel). (Ao: Aorta; LA: Left atrium; LV: Left ventricle).
Source: Dr. Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo
General Medical Center, Buffalo, NY (Movie clip 70.17). Transesophageal echo movie clip showing a papillary fibroelastoma attached to the
aortic valve.
Source: Dr Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo
General Medical Center, Buffalo, NY.
pedicled65 with a short stalk attached to the valve. Fingerlike projection on the fibroelastoma produce a prominent
fluttering appearance of the tumor surface, which is a
distinguishing feature on real time 2D TTE.
These tumors rarely exceed 1 cm in diameter and are
usually attached by a small pedicle to one of the cardiac
valve leaflet edges. Other less common anatomical sites
for PFE origin include: right atrium, RV, left atrium, LV,
left ventricular outflow tract (LVOT), atrial or ventricular
septum, and coronary ostia.69 Doppler echocardiography
usually demonstrates either minimal or mild regurgitation
but rarely significant regurgitation or stenosis.70,71
TTE has a sensitivity and specificity of 88.9% and 87.8%,
respectively, with an overall accuracy of 88.4% for the detection
of PFE 20 mm. An overall TTE sensitivity of 61.9% is reported
in case of tumor dimension 20 mm.65 A higher sensitivity is
reached with TEE, particularly for smaller PFEs.65 3D TTE has
also been successfully employed in PFE diagnosis.10,72,73
1477
Figs 70.18A to C: (A) Enlarged two-dimensional (2D) echocardiogram apical four-chamber view; (B) two-dimensional transesophageal echocardiogram (2D TEE), and (C) three-dimensional
transesophageal echocardiogram (3D TEE) showing a papillary
fibroelastoma attached to the chordal apparatus of the mitral valve.
(LA: Left atrium; LV: Left ventricle; RV: Right ventricle). Source:
Dr Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo General
Medical Center, Buffalo, NY (Movie clip 70.18A). 2D echo movie
clip showing a papillary fibroelastoma attached to a chordal
apparatus of the mitral valve.
Source: Dr. Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo
General Medical Center, Buffalo, NY (Movie clips 70.18A and
70.18B). 2D TEE movie clip showing a papillary fibroelastoma
attached to a chordal apparatus of the mitral valve. Source: Dr
Hashmat Ashraf, Chief of Cardiac Surgery, Buffalo General Medical
Center, Buffalo, NY.
Fig. 70.20: Live/real time three-dimensional transthoracic echocardiographic evaluation of tricuspid valve fibroelastoma. The arrow shows a
fibroelastoma attached to the septal leaflet (S) of the tricuspid valve. (AO:
Aorta; LA: Left atrium; RV: Right ventricle) (Movie clip 70.20).
Source: Reproduced with permission from Pothineni KR,
Duncan K, Yelamanchili P, et al. Live/real time three-dimensional
transthoracic echocardiographic assessment of tricuspid valve
pathology: incremental value over the two-dimensional technique.
Echocardiography. 2007;24(5):54152.
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Figs 70.21A to F: Pulmonary valve fibroelastoma. (A) Two-dimensional transthoracic echocardiography. Arrowhead points to fingerlike projections on the fibroelastoma; (B to E) Live/real time three-dimensional transthoracic echocardiography; (B) Arrowhead points to
fibroelastoma and the arrow to the echogenic stalk attaching it to the pulmonary valve (PV); (C) Cropping and sectioning the tumor and
viewing it en face shows no evidence of echolucency; (D) Arrowhead points to finger-like projections (fronds) on the fibroelastoma; (E)
Ex vivo imaging of the resected fibroelastoma shows a dense central core and multiple finger-like projections (arrowheads); (F) Gross
pathological specimen of fibroelastoma showing multiple frond-like structures resembling a sea anemone (left) and histopathology of the
surgical specimen demonstrating a central core of collagen and elastin covered by a single layer of endothelial cells (right). (AO: Aorta;
AV: Aortic valve; LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle; RVO: Right ventricle outflow tract) (Movie clips 70.21B,
70.21C Parts 1 and 2, and 70.21E). (Source: Reproduced with permission from Singh A, Miller AP, Nanda NC, Rajdev S, Mehmood F,
Duncan K. Papillary fibroelastoma of the pulmonary valve: assessment by live/real time three-dimensional transthoracic echocardiography. Echocardiography. 2006;23(10):8803.
1479
Figs 70.22A and B: Aortic valve fibroelastoma. Two-dimensional transesophageal echocardiography. Parasternal long-axis (A) and
short-axis (B) views. Arrowhead in A points to a mobile rounded mass attached to the right coronary cusp (arrow in B). The mass has
prominent, short, discrete projections on the surface, resembling a sea urchin or fronds of a curtain, and an echogenic central area from
collagen deposition. These findings are typical of a fibroelastoma. (AO: Aorta; LA: Left atrium; LAA: Left atrial appendage; RA: Right
atrium; RV: Right ventricle) (Movie clip 70.22).
Figs 70.23A and B: Tricuspid valve fibroelastoma. Two-dimensional transthoracic echocardiography. Right ventricular inflow (A) and
parasternal short-axis; (B) views show an echodensity with small, multiple spicule-like structures typical of a fibroelastoma (arrowhead).
(AV: Aortic valve; RA: Right atrium; RV: Right ventricle) (Movie clips 70.23A and B).
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.24A and B: Two-dimensional transesophageal echocardiography. Lambls excrescence. Arrowhead points to a Lambls
excrescence on an aortic (AO) valve cusp. (LA: Left atrium; LV: Left ventricle; RV: Right ventricle) (Movie clip 70.24).
Cardiac Fibroma
Benign cardiac fibromas, when diagnosed, are seen
almost exclusively in the pediatric population.79 Adult
cases have been reported but are exceedingly rare.80,81 It is
the second most common primary ventricular neoplasm
after rhabdomyomas. Grossly, these tumors are firm and
nonencapsulated, intramural lesions ranging in size from
3 cm to >10 cm in diameter.22
Clinically, cardiac fibromas may present as shortness
of breath, dizziness, syncope, and heart failure. Symptoms
occur when the tumor increases in size causing obstructive
disease. Cardiac fibromas may also be responsible for
asymptomatic murmurs since childhood, or be an origin
for arrhythmias. Fifty percent are asymptomatic. Cardiac
fibromas can be found incidentally as calcification in
lower mediastinum on thoracic spine radiograph, or on
CT of the chest. They most commonly occur in the RV
(Figs 70.25A to C; Movie clips 70.25AB, CBC)7 and are
Cardiac Rhabdomyoma
Cardiac rhabdomyoma is the most common primary
cardiac tumor in pediatric age group.87,88 This tumor is
C
multicentric and can occur in the ventricular free wall and
interventricular septum.8890 It occurs in equal frequency
in the right and left ventricles.
Diagnosis can occur in the prenatal period; however,
the majority are diagnosed in infancy. The incidence of
tuberous sclerosis in patients with cardiac rhabdomyoma
has been reported to be between 30 and 50%.91 Clinically,
rhabdomyoma can lead to life-threatening ventricular
arrhythmia or obstruction of ventricular filling or outflow
with intracavitary extension seen in 50% of patients.
Echocardiography is an ideal tool to diagnose
rhabdomyomas. These tumors tend to appear as multiple
homogenous, well-circumscribed, echogenic ventricular
masses. They may be intracavitary or intramural masses in
RV and LV. They have been described as having a speckled
appearance. A pedunculated rhabdomyoma occupying or
protruding into the ventricular cavity and aortic valve with
significant LVOT obstruction has been reported.92
1481
Cardiac Lipomas
Lipomas of the heart are most frequently located in the
LV (Figs 70.26A to C; Movie clips 70.26ABCAC), RV
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C
(Figs 70.27A and B; Movie clips 70.27 (Parts 1 to 3),7
and right atrium (Figs 70.28A to C; Movie clip 70.28).22
Clinically, these tumors are silent, although conduction
disturbances have been reported.95
Subepicardial tumors are large, often pedunculated,
whereas subendocardial lipomas are typically sessile.
About 25% are completely intramyocardial.22 Valvular
lipomas are rare, and have been described on the mitral
and tricuspid valves.96
Lipomas are differentiated from left ventricular
myxomas in that lipomas are less mobile and generally
more echodense. These tumors need to be differentiated
from lipomatous hypertrophy of the interatrial septum
(Figs 70.29A and B), which is characterized by accumulation
of adipose tissue in the atrial septum.97 In the subcostal views,
globular thickening of the interatrial septum with central
sparing gives what has been described as a dumbbell
shape to the atrial septum with lipomatous hypertrophy.
Figs 70.26A to C: Left ventricular lipoma. Live/real time threedimensional transthoracic echocardiography. The arrowheads
in A and B point to a highly echogenic mass in the left ventricle
(LV). Further cropping of the three-dimensional data set shows
the presence of two lipomas (arrowheads in C) in the left ventricle.
Arrow in the movie clips 70.26A and B points to the lipoma that
is highly echogenic and shows no echolucencies on cropping.
Movie clip 70.26C shows another patient with a left ventricular
lipoma (arrow) together with the surgically resected specimen.
A few trabeculations are also present adjoining the lipoma in
the left ventricular apex. (RV: Right ventricle). (Movie clips
70.26AB, C).
Cardiac Hemangiomas
Hemangiomas are the most common benign vascular
tumors of the heart (Figs 70.30A to D; Movie clip 70.30 B).8
These tumors are usually solitary but may be multiple.99
They may occur anywhere in the heart including the
epicardium, often in association with pericardial effusion.99
They may be either intracavitary or intramural. Intramural
hemangiomas occur most commonly on the right side of
the heart (Figs 70.31A to D; Movie clips 70.31A, B, and D
Parts 12),34 particularly in the interventricular septum.99
Clinical signs depend on anatomical location.
Congestive heart failure occurs with large intracavitary
1483
Figs 70.27A and B: Right ventricular lipoma. Live/real time three-dimensional transthoracic echocardiography. (A) The arrow points to
a large markedly echogenic lipoma interdigitating and infiltrating the right ventricle (RV) free wall. (B) Surgical specimen. (LA: Left atrium;
LV: Left ventricle) (Movie clips 70.27 Parts 1 to 3).
Source: Reproduced with permission from Reddy VK, Faulkner M, Bandarupalli N, et al. Incremental value of live/real time three-dimensional
transthoracic echocardiography in the assessment of right ventricular masses. Echocardiography. 2009;26(5):598609.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.29A and B: Two dimensional transesophageal echocardiography. Lipomatous hypertrophy of the atrial septum. (A) Arrow
points to thickening of atrial septum that spares foramen ovale. This is caused by fatty infiltration of the septum and usually has no
clinical sequelae. (B) Massive lipomatous hypertrophy (arrowheads) affects the entire atrial septum and occupies most of the right
atrium (RA) in another patient. (LA: Left atrium; RA: Right atrium; RPA: Right pulmonary artery; SVC: Superior vena cava). (Courtesy
of Dr Allan Schwadron, Dothan, AL).
1485
Figs 70.30A to D: Hemangioma involving the mitral valve. (A) Two-dimensional transthoracic echocardiography. Parasternal long-axis
view. The arrow points to the mass attached to the anterior mitral leaflet (AML), with a few echolucencies within it; (B) Live/real time
three-dimensional transthoracic echocardiography. Apical four-chamber view. The arrow points to a mass attached to the mitral valve.
The presence of multiple echolucencies (arrowheads, inset A) within the mass is consistent with a hemangioma; (C) Two-dimensional
transesophageal echocardiography. Low esophageal modified five-chamber view. The arrow points to the mass on the AML, with a few
scattered echolucencies within it; (D) Histopathology. H&E stain 10x. In addition to the sheet-like areas of epithelioid endothelial cells
with small vascular spaces typical of epithelioid hemangioma (arrows), this lesion also contains larger, more cavernous vascular spaces
(arrowheads). (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle). (Movie clip 70.30B).
Source: Reproduced with permission from Dod HS, Burri MV, Hooda D, et al. Two- and three-dimensional transthoracic and transesophageal echocardiographic findings in epithelioid hemangioma involving the mitral valve. Echocardiography. 2008;25(4):4435.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.31A to D: Right ventricular hemangioma. (A) Two-dimensional transesophageal echocardiography. The tumor (T) is located
in right ventricle (RV); (B to D) Live/real-time three-dimensional transesophageal echocardiography; (B) The tumor (arrow) is located
in RV; (C) Arrow points to multiple small echolucencies involving the whole tumor including the periphery, typical of hemangioma;
(D) The three-dimensional data set was cropped from bottom and rotated to view the echogenic area of tumor attachment (dotted lines).
It measured 2.76 1.40 cm, area 2.01 cm2. (AV: Aortic valve; LV: Left ventricle; PA: Pulmonary artery; RA: Right atrium; TV: Tricuspid
valve) (Movie clips 70.31A, B, D Parts 1 and 2).
Source: Reproduced with permission from Khairnar P, Hsiung MC, Mishra S, et al. The ability of live three-dimensional transesophageal
echocardiography to evaluate the attachment site of intracardiac tumors. Echocardiography. 2011;28(9):10415.
1487
Figs 70.32A and B: Left atrial hemangioma. Live/real time threedimensional transthoracic echocardiography. (A) Arrowheads
(arrow in Movie clip 70.32) point to two of the large number of
closely packed echolucencies in the tumor mass with sparse solid
tissue; (B) Resected specimen showing multiple vascular areas.
(LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RA: Right
atrium; RV: Right ventricle) (Movie clip 70.32).
Source: Reproduced with permission from Mehmood F, Nanda
NC, Vengala S, et al. Live three-dimensional transthoracic echocardiographic assessment of left atrial tumors. Echocardiography.
2005;22(2):13743.
Fig. 70.33: Right ventricular sarcoma. Live/real time threedimensional transthoracic echocardiography. Arrow points to a
mass in the right ventricle (RV) showing large echolucencies
surrounded by echogenic band-like tissue giving a doughnut
appearance. Movie clip 70.33B shows, in a different patient, renal cell
carcinoma invading the inferior vena cava (IVC) and the proximal right
atrium (RA). Cut section demonstrates a solid tumor with no evidence
of necrosis. Surgically resected specimen is also shown. (LA: Left
atrium; LV: Left ventricle) (Movie clips 70.33A and B).
Source: Reproduced with permission from Reddy VK, Faulkner M,
Bandarupalli N, et al. Incremental value of live/real time three-dimensional transthoracic echocardiography in the assessment of right ventricular masses. Echocardiography. 2009;26(5):598609.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
B
Figs 70.34A to C: Left atrial leiomyosarcoma. (A) Live/real time
three-dimensional transthoracic echocardiography. The arrows
point to a doughnut-like appearance of the tumor mass located
in the left atrium; (B) Intermediate and high-power microscopic
appearances of the leiomyosarcoma. The tumor is composed
of pleomorphic spindle cells arranged in a fascicular pattern.
Abnormal mitotic activity (inset) was easily identified; (C) Lowpower microscopic appearance of necrotic tumor. The periphery
of a necrotic area within the tumor showed acellular stroma
and dilated/ectatic blood vessels (arrows) consistent with the
echocardiographic findings. (AO: Aorta; LA: Left atrium; LV: Left
ventricle) (Movie clip 70.34).
Source: Reproduced with permission from Suwanjutah T, Singh H,
Plaisance BR, Hameed O, Nanda NC. Live/real time three-dimensional transthoracic echocardiographic findings in primary left atrial
leiomyosarcoma. Echocardiography. 2008;25(3):3379.
Figs 70.35A to F
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.35A to H: Aortic leiomyosarcoma in a 43-year-old male. (A to F) A mass (arrowheads) in the descending thoracic aorta (DA).
Color Doppler examination shows prominent flow signals in the unobstructed portion of the aorta (AO). This makes thrombus unlikely,
because associated spontaneous contrast echoes caused by a low-flow state usually are present. Also, no dissection flap is identified.
The arrows in A to C and E to H show a large echogenic mass outside the AO, consistent with hematoma; (G and H) A hematoma
(arrow, arrowheads) is seen extending on both sides of the descending aorta (DA, AO), even where the tumor mass is not present. At
surgery, the mass was found to be a leiomyosarcoma that involved the aortic wall, resulting in perforation that caused the hematoma
(Movie clip 70.35).
Source: Reproduced with permission from Suwanjutah T, Singh H, Plaisance BR, Hameed O, Nanda NC. Live/real time three-dimensional transthoracic echocardiographic findings in primary left atrial leiomyosarcoma. Echocardiography. 2008;25(3):3379.
Cardiac Plasmacytoma
Primary plasmacytoma of the heart is extremely rare.
These tumors, when present in the heart, are usually
secondary to metastatic disease or direct extension from
the mediastinum. The right atrium is the most common
site of cardiac plasmacytoma.140 They also can originate in
the atrioventricular groove with a short stalk perforating
into the right atrium near the tricuspid valve annulus.
Heart failure is the most common clinical presentation.
Plasma immunoelectrophoresis detection of monoclonal
immunoglobulin G paraproteinemia and k light chains
increases the probability for this disease entity. Bence
Jones proteinuria may or may not be present. On gross
1491
Figs 70.36A and B: Two-dimensional transesophageal echocardiography. Hodgkin lymphoma involving the atrial septum. (A) Fourchamber view demonstrating thickening of the atrial septum (arrowhead) produced by the tumor. Note sparing of the fossa ovalis;
(B) Arrowhead shows a large circumscribed echolucent area in the atrial septum containing multiple, bright echo densities (coin lesion).
(LA: Left atrium; MV: Mitral valve; RA: Right atrium; SVC: Superior vena cava; TV: Tricuspid valve).
Source: Reproduced with permission from Miller A, Mukhtar O, et al. Two- and three-dimensional TEE differentiation of lymphoma
involving the atrial septum from lipomatous hypertrophy. Echocardiography. 2001;18(3):2059.
Hydatid Cyst
Cardiac hydatid cyst is a rare parasitic disease caused by
larvae of Echinococcus granulosus, which is still endemic
in many sheep-raising countries. Although the LV is mostly
involved in this condition, all cardiac chambers and rarely
the pericardium can be affected (Figs 70.38A to Z; Movie
clips 70.38A and B; Figs 70.39A to F). Patients can be
asymptomatic, have nonspecific symptoms such as chest
pain, shortness of breath, or palpitations or present as an
acute complication of cyst rupture.142 Echocardiogram
usually shows a unique finding of multiple cysts inside the
cardiac chambers or the pericardium.
Pericardial Mesotheliomas
Primary pericardial mesothelioma is a very rare tumor,
with a reported prevalence of <0.002%.143 In addition to
1492
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.38A to F
1493
1494
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.38G to L
Figs 70.38M to R
1495
1496
Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.38S to X
1497
Figs 70.38A to Z: Live/real time three-dimensional transthoracic echocardiography. Hydatid cyst. Arrow in the apical four-chamber (A)
and short-axis (B) views in a young Asian Indian policeman shows a large mass in the left ventricle (LV), which represents a hydatid cyst
that had become smaller since the previous study done a few years ago. This collapse of the hydatid cyst could have been spontaneous or may be related to antihelminthic albendazole therapy, which had been given to this patient. (LA: Left atrium; RV: Right ventricle)
(Movie clips 70.38A and B). (C to Z) Live three-dimensional transthoracic echocardiographic assessment of hydatid cyst in the left ventricle in another patient a 37-year-old Asian Indian male. (C) The arrow points to the large hydatid cyst in the left ventricle (LV) seen on
the two-dimensional transthoracic echocardiogram. (B to I) Sequential transverse plane (TP) sections taken at various levels (# 15) of
the three-dimensional data set. The cyst is not visualized at the level of the body of the mitral valve(C, *), but its basal tip (arrow) comes
into view when the section is taken further down at the mitral valve leaflet tips (D). In H and I, the large arrowhead points to a tertiary or
grand-daughter cyst located within the secondary or daughter cyst. The small arrowhead in H shows a small greatgrand-daughter cyst
budding from the tertiary cyst. The arrow points to the parent hydatid cyst. (J to M) Longitudinal plane (LP) sections (# 1 and 2) through
the hydatid cyst (arrow). (N) Combined TP and LP sections through the hydatid cyst. (O to T) Only FP (O), combined FP and TP (P and
Q), combined FP and LP (R), and combined FP, TP, and LP (S and T) sections through the hydatid cyst. (U to Y) Oblique plane (OP)
sections through the hydatid cyst. In X, the OP section is rotated to view the cyst en face. A tertiary or grand-daughter cyst is shown
attached to the secondary or daughter cyst by a stalk (small double arrowheads). (Z) Schematic of hydatid cyst. (LA: Left atrium; RA:
Right atrium; RV: Right ventricle; S: Stalk). (Movie clips 70.38CZ).
(Source: Reproduced with permission from Sinha A, Nanda NC, Panwar R, et al. Live three-dimensional transthoracic echocardiographic assessment of left ventricular hydatid cyst. Echocardiography. 2004;21(8):699705.
A
Figs 70.39A and B
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.39A to F: (A) Transthoracic echocardiography shows a large pericardial hydatid cyst (MS) compressing the left ventricular (LV)
lateral wall. It measures approximately 8 cm in diameter and appears to contain layered solid tissue. The patient was an 18-year-old
Asian Indian male who had emigrated to South Alabama at the age of 5 from India. He presented with a recent syncopal episode and the
electrocardiography (ECG) showed deeply inverted T-waves in anterolateral leads consistent with myocardial ischemia. Initially, the twodimensional echocardiogram appeared to show poor LV function but it soon became apparent that this was artifactual and resulted from
the ultrasonic beam passing directly from the aortic root into the cyst; (B and C) The arrows point to multiple intramyocardial coronary
vessels imaged within the compressed LV free wall. These were visualized using a high-resolution color Doppler system. Note that the
Nyquist limit is set at a very low velocity of 0.16 m/s; (D) The arrows demonstrate a high velocity of 1.2 m/s obtained by color Dopplerguided pulsed Doppler interrogation of an intramyocardial coronary vessel (normal velocity < 0.6 m/s). Thus, there was compression
of the LV lateral wall by the cyst that served to explain the ECG changes. A coronary arteriogram also showed systolic emptying of the
first marginal branch of the circumflex artery. The cyst was surgically resected and the patient is doing well; (E) Left: Three-dimensional
reconstruction demonstrates membrane-like solid tissue in the cyst. Right: Three-dimensional reconstruction of intramyocardial coronary vessels (arrows) in the compressed LV wall; (F) Left upper panel: Hydatid cyst wall and surrounding soft tissue. Left lower panel:
Close-up of hydatid scolex with double rows of hooklets (100x). Right panel: Laminated membrane. (C: Pericardial cyst; LA: Left atrium;
RA: Right atrium; RV: Right ventricle) (Movie clip 70.39).
Source: Reproduced with kind permission from Kluwer Academic Publishers, Advances in Echo Imaging using Contrast Enhancement, 2nd edition, 1997, Ch. 41 Echocardiographic detection of intramyocardial coronary obstruction produced by pericardial hydatid
CPT, Fig. 2B. Jamil F, Nanda NC, et al. Echocardiographic Detection of Intramyocardial Coronary Obstruction Produced by Pericardial
Hydatid Cyst. Echocardiography. 1997;14(5):45960.
1499
Fig. 70.41: Apical four-chamber view showing metastatic melanoma with metastasis to the papillary muscle. (LA: Left atrium; LV:
Left ventricle; RA: Right atrium; RV: Right ventricle).
Figs 70.42A and B: Two-dimensional transesophageal echocardiography. Metastatic melanoma involving the right ventricle. (A and
B) A huge tumor mass (M, T) is noted in the right ventricle (RV) reaching up to the pulmonary valve. (AO: Aorta; LV: Left ventricle; PA:
Pulmonary artery).
Source: Reproduced with permission from Nanda NC, Domanski MJ, editors. Atlas of Transesophageal Echocardiography. 2nd ed.
Philadelphia, PA: Lippincott Williams & Wilkins; 2007:519.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
and (d) by intraluminal venous extension. About twothirds of all cardiac metastases involve the pericardium,
one-third the epicardium or the myocardium, and only 5%
the endocardium.148
Tumors such as the bronchial, breast, and esophageal
carcinoma, which develop near the heart, may expand by
direct extension into the heart, but predominantly by the
lymphatics. All preferentially affect the pericardium.148153
In the case of pericardial involvement, echocardiography
can show dense pericardial bands reflecting thickening
by inflammation or tumor infiltration. Pericardial effusion
can be proven quickly, with high sensitivity. When the
pericardium is involved, echocardiography is usually
used both as a guidance for pericardiocentesis and for the
follow-up of effusions. Tumor cells within the pericardial
fluid may verify diagnosis of metastatic pericardial
involvement (see Figs 70.43 and 70.44).
Malignant melanoma (see Figs 70.41 and 70.42),
lymphoma (see Fig. 70.43), leukemia, soft tissue, and bone
sarcoma usually spread hematogenously. Myocardial
metastases can involve any one of the heart chambers
(Figs 70.45A to E, Movie clips 70.45B, C and DE; Figs
70.46A to E; Movie clips 70.46A and B).13,159 Intracavitary
cardiac metastases are the least common variety. Echocardiography cannot distinguish intracavitary metastatic
tumor from primary cardiac tumors.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.46A to E: Metastatic chordoma. (A) Real-time twodimensional transthoracic echocardiography. The arrow points to
the chordoma in the right ventricle (RV); (B and C) Live/real time
three-dimensional transthoracic echocardiography. Arrows point
to echolucencies consistent with hemorrhages and cystic areas,
and the arrow heads denote echodense areas produced by fibrotic bands. The less intense areas represent the myxoid stroma;
B and C represent in-vivo and ex-vivo studies, respectively;
(D and E) Pathological specimen showing the lobulated resected
tumor (D); The cut surface (E) shows hemorrhagic and cystic areas
(arrows) and dense fibrotic bands (arrow heads). (LA: Left atrium;
RA: Right atrium; TV: Tricuspid valve) (Movie clips 70.46A and
70.46B).
Source: Reproduced with permission from Pothineni KR, Nanda
NC, Burri MV, Bell WC, Post JD. Live/real time three-dimensional
transthoracic echocardiographic description of chordoma metastatic to the heart. Echocardiography. 2008;25(4):4402.
Chiari Network
The Chiari network is an embryological remnant that results
from incomplete reabsorption of the right valve of the sinus
venosus. It appears as a web-like structure with thread-like
components and is present in 2% of the population. Its widely
mobile and serpiginous appearance within the right atrium
can be confused with other highly mobile pathological
structures including vegetations, flail tricuspid leaflet,
ruptured chordae tendinae, thrombus, or right heart tumor.46
The key differential points by echocardiogram include (a)
identification of two, and ideally three, normal appearing
tricuspid valve leaflets; (b) presence of a bright, rotatory,
highly mobile echocardiographic target that does not move
into the right ventricular inflow tract or RV in diastole as
would be typical of a tricuspid leaflet vegetation; and (c)
in the four-chamber apical or subcostal view, the typical
posterolateral orientation and anteroinferior and medial
course of this structure across the right atrium. The use of
intravenous bolus contrast material to outline the course of
the IVC, right atrium, tricuspid valve, and RV can also be of
additional benefit in excluding the presence of RA mass and
tricuspid leaflet disruption.46
Eustachian Valve
The Eustachian valve, an embryological remnant of the
valve of the IVC, commonly appears as a thin flap arising
from the anterior rim of the IVC orifice. However, it can also
persist as a mobile, elongated structure that projects several
centimeters into the RA cavity and has been misinterpreted
as an intra-atrial thrombus. Eustachian valves move in a
more restricted manner than Chiari networks, and should be
imaged from the apical four-chamber view, right inflow tract,
and subcostal views to distinguish them from vegetation,
tumor, or thrombus in the right atrium. On echocardiogram,
it appears as a linear, echodense object.
Thebesian Valve
The thebesian valve, also known as the valve of the
coronary sinus, is a semicircular fold of the lining
1503
Crista Terminalis
The crista terminalis is a well-defined fibromuscular
structure formed by the regression of the septum
spurium as the sinus venosus is incorporated into the
RA wall. Depending on the amount of regression, the
crista terminalis can also be very prominent and mimic
a RA mass. On TTE, the crista terminalis is seen as an
echodense linear ridge in the posterior RA wall, extending
laterally from the atrial septum. On 3DE, it can be more
clearly defined as a thick and tapering linear structure in
the posterior wall of the right atrium.16
Moderator Band
The moderator band, or septomarginal trabeculation,
is a muscular trabeculation extending from the lower
interventricular septum to the anterior RV wall. It is easily
identified because of its location in the RV, carrying the
right bundle branch from the bundle of His. It is best seen
in the apical four-chamber view and subcostal views, and
in the apical view, can be seen as it transverses the RV
cavity at midventricular level, connecting the free wall to
the interventricular septum. In the parasternal long-axis
view of the RV, it can be seen as a large muscular band that
crosses the apex of the RV obliquely.
Coumadin Ridge
The Q-tip sign, also known as the Coumadin ridge, is
a prominent muscular structure formed between the left
atrial appendage (LAA) and the atrial insertion of the left
upper pulmonary vein. It was often misdiagnosed as a
thrombus until it became well described in the literature.
It can be differentiated from a thrombus by its lack of
mobility and characteristic location. This tissue may
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Thrombus
Cardiac thrombi can be located within the atrial (Figs
70.47A and B, Movie clips 70.47, Figs 70.48A to E)7 and
ventricular chambers (Figs 70.49 and 70.50),7 and it can
extend to the heart from the vena cava (Figs 70.51A to
C) or from the heart to the pulmonary arteries (Figs 70.51A
to C and 70.52). They may develop as a consequence of
multiple underlying cardiac disorders affecting the valves
and myocardium.
Echocardiography serves as a cornerstone in the
evaluation and diagnosis of these patients. It is considered
to be the first-line imaging modality in such patients.
Atrial Thrombus
Within the atria, thrombi can be found in the atrial appendages, within the body of the atrium, or in a combination
of these areas. They are usually the consequence of poor
atrial emptying and blood stasis in conditions such as atrial
fibrillation, atrial flutter, and mitral stenosis. They may also
be associated with indwelling catheters (see Figs 70.51A to
C). The most common location for thrombi in the atrium
is the LAA, which cannot be regularly seen by TTE. In this
Figs 70.47A and B: Live/real time three-dimensional transthoracic echocardiographic assessment of right atrial thrombus. (A)
Arrowhead points to a large mobile serpiginous thrombus in the right atrium (RA) prolapsing into the right ventricle (RV); (B) Cropped
segments of the thrombus (arrowheads) demonstrate a homogenous appearance with no echolucencies, indicating absence of clot lysis
(Movie clip 70.47).
Source: Reproduced with permission from Nanda NC, Hsiung MC, Miller AP, Hage FG: Live/Real Time 3D Echocardiography. Oxford,
UK: Wiley-Blackwell; 2010.
1505
E
Figs 70.48A to E: Live/real-time three-dimensional transthoracic echocardiography. (A and B) Apical (A) and parasternal short-axis
(B) views show a large mass (arrow) consistent with thrombus in the right atrium (RA) with possible attachment to the RA free wall;
(C and D) Right parasternal views showing the attachment of the thrombus to the RA free wall in the vicinity of the inferior vena cava
(IVC) and tricuspid valve (TV) directly opposite the superior vena cava (SVC) entrance into the RA. (LA: Left atrium; LV: Left ventricle;
RV: Right ventricle); (E) Schematics of (a) clot, (b) myxoma, (c) sarcoma/chordoma, and (d) hemangioma. The horizontal arrowhead
in (a) points to central lysis in a clot, in (b) it points to an area of hemorrhage/necrosis in a myxoma, and in (c) to an area of necrosis
surrounded by thick, band-like tissue containing collagen, giving a doughnut-like appearance seen with chordoma and sarcoma. The
vertical arrowhead in (b) points to dense calcification in the myxoma. (d) Demonstrates a hemangioma that is completely vascular and
the echolucencies involve the whole tumor, including periphery (Movie clips 70.48AD).
Source: Reproduced with permission from Reddy VK, Faulkner M, Bandarupalli N, et al. Incremental value of live/real time three-dimensional
transthoracic echocardiography in the assessment of right ventricular masses. Echocardiography. 2009;26(5):598609.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.49A to D: Two-dimensional (A) and live/real-time three-dimensional (B to D) transthoracic echocardiography in right ventricular
thrombus. (A) The arrowhead points to one bifid or possibly two clots in the right ventricle (RV). (B and C) Cropping the three-dimensional (3D) data set demonstrates three separate clots in the RV (arrowheads). (D) Another patient with clots in RV showing central lysis in
a cropped 3D image. (LA: Left atrium; LV: Left ventricle; RA: Right atrium) (Movie clips 70.49A, BC parts 1 to 4, D).
Source: Reproduced with permission from Reddy VK, Faulkner M, Bandarupalli N, et al. Incremental value of live/real time
three-dimensional transthoracic echocardiography in the assessment of right ventricular masses. Echocardiography. 2009;26(5):598
609.
Vegetation
Cardiac vegetation presents as an oscillating or nonoscillating intracardiac mass attached to the valves, other
endocardial structures, or implanted on intracardiac
material. It is considered to be the pathological hallmark
of endocarditis. Endocarditis can involve any of the heart
1507
Figs 70.50A to D: Live/real-time three-dimensional transthoracic echocardiogram. (A) Four-chamber view showing no thrombus or
aneurysm in the left ventricle (LV); (B) Anteriorposterior cropping displays the large aneurysm containing thrombus (arrow); (C and D)
Sectioning of the thrombus (C) and viewing it en face (D) shows no evidence of lysis or liquefaction. (RV: Right ventricle) (Movie clip
70.50).
Source: Reproduced with permission from Duncan K, Nanda NC, Foster WA, Mehmood F, Patel V, Singh A. Incremental value of live/
real time three-dimensional echocardiography in the assessment of left ventricular thrombi. Echocardiography 2006;23(1):6872.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.51A to C: Two-dimensional transesophageal echocardiography. Thrombus in the pulmonary artery and the superior
vena cava. (A to C) Large thrombi (M,C) are noted in the superior vena cava (SVC) and the right pulmonary artery (RPA) in this
patient with an infusion catheter, which acted as the nidus for
thrombus. (AO: Aorta; ASC AO: Ascending aorta; LA: Left atrium).
Source: Reproduced with permission from Nanda NC, Domanski
MJ, editors. Atlas of Transesophageal Echocardiography. 2nd ed.
Philadelphia: Lippincott Williams & Wilkins; 2007:521.
1509
D
Figs 70.52A to E: Two-dimensional transesophageal echocardiography. A 78-year-old black female. (A and B) Thrombic obstruction
of distal left and right pulmonary arteries. Arrowhead points to a
large thrombus in the distal left pulmonary artery (LPA, A) and in
the distal right pulmonary artery (RPA, B); (C to E) Thrombotic
occlusion of LPA descending lobar branches; (C) Large arrowhead
points to the thrombus (TH) and small arrowhead points to an
echolucent area of clot lysis. Arrow shows extension of the TH into
a descending lobar branch of the LPA; (D) Turbulent flow signals
with prominent flow acceleration are noted in a descending lobar
branch (labeled 2). Arrow points to the left pulmonary vein (LPV),
and arrowhead shows the TH; (E) Color Doppler-guided continuous
wave Doppler interrogation (arrow) shows a high systolic velocity
of 2.2 m/s and a high diastolic velocity of 1.0 m/s indicative of
significant obstruction. (AO: Aorta; T: Transverse plane) (Movie
clips 70.52A to E).
Source: Reproduced with permission from Kang SW, Nekkanti R,
Nanda NC, et al. Transesophageal echocardiographic identification of thrombus producing obstruction of left pulmonary artery descending lobar branches and bronchial artery dilatation. Echocardiography. 2002;19:838.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.53A to D: Transesophageal echocardiographic finding of LAA lobe mimicking a mass lesion. (A) The arrowhead points to what
appears to be a mass in or adjacent to LAA; (B) Schematic; (C) Keeping the mass-like lesion in the middle of the monitor screen and
rotating the transducer from 0 to 180 shows that the mass-like effect is produced by a lobe (#3) of the LAA; (D) Schematic. Numbers
1 and 2 denote the other two lobes of the LAA. (AO: Aorta; LA: Left atrium; LAA: Left atrial appendage; LV: Left ventricle; M: Mass; MV:
Mitral valve; PA: Pulmonary artery; PE: Fluid in the transverse sinus of pericardium) (Movie clip 70.53).
Source: Reproduced with permission from Giove GC, Singla I, Mishra J, Nanda NC. Transesophageal echocardiographic finding of left
atrial appendage lobe mimicking a mass lesion. Echocardiography. 2011;28:6845.
C
increasing paravalvar regurgitation or valve dehiscence is
a major criterion for the diagnosis of endocarditis.
Extension of the infection to the perivalvar tissues is
a sign of poor prognosis in the evolution of the disease.
Extravalvar extension may lead to endothelial erosion,
perivalvar abscess, mycotic aneurysm, and intracardiac
fistulae.
Perivalvular Abscess
Perivalvar cavities are formed when annular infections
break through and spread into contiguous tissue. In native
aortic valve endocarditis, they generally occur through
the weakest portion of the annulus, which is near the
membranous septum. Perivalvar abscesses are particularly
common in prosthetic valve endocarditis, since the
annulus is the usual primary site of infection. A perivalvar
1511
MICE
Mesothelial/macrophage incidental cardiac excrescences,
or MICE, are made up of mesothelial cells, macrophages,
scattered inflammatory cells and fibrin, and lack a vascular
network or supporting stroma.172 Lack of vascularity is an
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.55A and B: Real time two-dimensional transthoracic echocardiography. Arrowhead points to a large echogenic mass involving
the posterior mitral annulus, consistent with calcification. An echolucent area consistent with liquefaction is seen in B. (AO: Aorta; LA:
Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle) (Movie clips 55A and B).
(Source: Reproduced with permission from Assudani J, Singh B, Samar A, et al. Echocardiography. 2010;27:114750).
Fig. 70.56: Live/real time three-dimensional transthoracic echocardiography. Cropped three-dimensional data set. The lower
arrowhead points to a highly echogenic calcification while the upper
arrowhead shows a less echogenic area of uniform appearance.
(MV: Mitral valve; TV: Tricuspid valve) (Movie clip 70.56).
Source: Reproduced with permission from Assudani J, Singh B,
Samar A, et al. Echocardiography. 2010;27:114750.
1513
Fig. 70.57: Real time two-dimensional transesophageal echocardiography. The arrowhead denotes posterior mitral annular
calcification. Note shadowing and reverberations beneath the
calcification. (LA: Left atrium; LV: Left ventricle) (Movie clip 70.57).
Source: Reproduced with permission from Assudani J, Singh B,
Samar A, et al. Echocardiography. 2010;27:114750.
Figs 70.58A to D: Live/real time three-dimensional transesophageal echocardiography in caseous mitral annular calcification.
(A and B) Four-chamber views. The arrowheads in A and B point to a large echogenic mass involving the posterior mitral annulus,
consistent with calcification. In B, the mass shows multiple, discrete, band-like, and punctuate echodensities surrounded by a highly
echogenic border. (C and D) Cropping of the four-chamber data set shows both a highly echogenic (lower arrowhead) and a relatively
less echogenic (upper arrowhead) components of the mass. Transverse cropping (D) also reveals a pattern of highly echogenic
component as well as a relatively less echogenic area with multiple, small, discrete band-like, and punctate echodensities. (AML:
Anterior mitral leaflet; AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle). [Movie clips 70.58A (Parts 1 and 2), B to D
(Parts 1 and 2)].
Source: Reproduced with permission from Assudani J, Singh B, Samar A, et al. Echocardiography. 2010;27:114750.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.59A and B: Two-dimensional transesophageal echocardiography. Extracardiac tumor. (A and B) Tumor (T, arrow) is seen posterior to the aortic root bulging into the left atrium (LA). (AO: Aorta; LV: Left atrium; RV: Right ventricle).
Source: Reproduced with permission from Nanda NC, Domanski MJ, editors. Atlas of Transesophageal Echocardiography. 2nd ed.
Philadelphia, PA: Lippincott Williams & Wilkins; 2007:524.
Extracardiac Masses
In addition to normal cardiac variants, certain extracardiac
masses can also mimic cardiac tumors by compressing
the cardiac chambers from the outside of the heart and
creating a mass effect. Examples include tumors or diseases
of the mediastinum (Figs 70.59 to 70.63), hematomas
(Fig. 70.64), coronary aneurysms, and pseudoaneurysms.
Although not uncommon, pericardial fat deposition
when prominent can masquerade as extracardiac tumor
(Figs 70.65A and B; Movie clips 70.65A and B).
Sclerosing mediastinitis is a rare disorder characterized
by the invasive proliferation of fibrous tissue within the
mediastinum and frequently results in the compression
of vital mediastinal structures including the heart. It can
cause compression of the atrial or ventricular cavities
and can cause obstruction of the SVC and the pulmonary
arteries (Figs 70.60 to 70.63).
Intracardiac Hardware
In addition to intrathoracic masses mimicking cardiac
tumors, intracardiac hardware such as Impella percutaneous left ventricular assist device catheters, pacing
leads, SwanGanz catheters, and central line catheters
are also commonly seen left atrial foreign bodies on
echocardiography. Catheters and pacemaker leads are
usually seen on echocardiography as two parallel thin
linear echodensities separated by an echolucent slit that
produce typical reverberations and side lobe artifacts
A
Figs 70.61A and B
1515
Figs 70.60A to C: Two-dimensional transesophageal echocardiography. Sclerosing mediastinitis. This 43-year-old man presented
with respiratory failure. (A) A mass (M) surrounds the left atrium
(LA), right pulmonary artery (RPA), and superior vena cava (SVC);
(B and C) The mass appears to infiltrate and invaginate into the LA
and extends up to the base of the left atrial appendage (LAA). This
resembles an intracardiac tumor. (AO: Aorta; AV: Aortic valve; PA:
Pulmonary artery; RA: Right atrium; RVO: Right ventricular outflow).
Source: Reproduced with permission from Kovach TA, Nanda NC,
Kim KS, et al. Transesophageal echocardiographic findings in sclerosing mediastinitis. Echocardiography. 1996;13:1038.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.61A to C: Two-dimensional transesophageal echocardiography. Sclerosing mediastinitis. Same patient as in Figures
70.60 A and B. Both the right lower pulmonary vein (RLPV) and
right upper pulmonary vein (RUPV) demonstrate obstruction near
their entrance in the left atrium (LA). The exact sites of obstruction
in the lower and upper pulmonary veins, shown by the arrow and
the arrowhead, respectively, mark the transition from laminar (red)
to disturbed (mosaic) flow; (C) Pulsed Doppler interrogation of
the mosaic flow reveals a high velocity of 2.58 m/s, indicative of
obstruction. (LV: Left ventricle; M: Mass; RVO: Right ventricular
outflow; SVC: Superior vena cava).
Source: Reproduced with permission from Kovach TA, Nanda NC,
Kim KS, et al. Transesophageal echocardiographic findings in
sclerosing mediastinitis. Echocardiography. 1996;13:1038.
Figs 70.62A to C: Two-dimensional transesophageal echocardiography. Sclerosing mediastinitis. Same patient as in Figures 70.60
and 70.61. (A and B). The arrow points to the site of obstruction in
the superior vena cava (SVC) near its junction with the right atrium (RA). Color Doppler examination shows a thin mosaic flow jet
in (B), indicative of obstruction; (C) Pulsed Doppler interrogation
reveals a high velocity of at least 1.61 m/s. (LA: Left atrium; M:
Mass; RPA: Right pulmonary artery).
Source: Reproduced with permission from Kovach TA, Nanda NC,
Kim KS, et al. Transesophageal echocardiographic findings in
sclerosing mediastinitis. Echocardiography. 1996;13:1038.
1517
Figs 70.63A and B: Sclerosing mediastinitis. Histology of mediastinal biopsy tissue from the same patient shown in Figures 70.60 to
70.62. (A) Photomicrograph of sclerosing process impinging on mediastinal adipose tissue (congo red, original magnification 125);
(B) Photomicrograph of collagenization of blood vessel wall with narrowing of the lumen (center), a region of cellular fibrosis (above),
and a region of acellular fibrosis (below; hematoxylin and eosin, original magnification 125).
Source: Reproduced with permission from Kovach TA, Nanda NC, Kim KS, et al. Transesophageal echocardiographic findings in
sclerosing mediastinitis. Echocardiography. 1996;13:103108.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
Figs 70.65A and B: Two-dimensional transthoracic echocardiography. Epicardial fat pad. Arrows point to prominent epicardial fat deposition both anteriorly and posteriorly visualized in parasternal long-axis view and around the cardiac apex in the apical four-chamber
view (Movie clips 70.65A and B). (LA: Left atrium; LV: Left ventricle; RV: Right ventricle).
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1. Plana JC. Three-dimensional echocardiography in the
assessment of cardiac tumors: the added value of the extra
dimension. Methodist Debakey Cardiovasc J. 2010;6(3):
1219.
2. Asch FM, Bieganski SP, Panza JA, Weissman NJ. Real time
3-dimensional echocardiography evaluation of intracardiac
masses. Echocardiography. 2006;23(3):21824.
3. Meng Q, Lai H, Lima J, et al. Echocardiographic and
pathologic characteristics of primary cardiac tumors: a
study of 149 cases. Int J Cardiol. 2002;84(1):6975.
4. Lokhandwala J, Liu Z, Jundi M, et al. Threedimensional echocardiography of intracardiac masses.
Echocardiography. 2004;21(2):15963.
5. Mehmood F, Nanda NC, Vengala S, et al. Live threedimensional transthoracic echocardiographic assessment
of left atrial tumors. Echocardiography. 2005;22(2):13743.
6. Mller S, Feuchtner G, Bonatti J, et al. Value of
transesophageal 3D echocardiography as an adjunct to
conventional 2D imaging in preoperative evaluation of
cardiac masses. Echocardiography. 2008;25(6):62431.
7. Reddy VK, Faulkner M, Bandarupalli N, et al. Incremental
value of live/real time three-dimensional transthoracic
echocardiography in the assessment of right ventricular
masses. Echocardiography. 2009;26(5):598609.
8. Dod HS, Burri MV, Hooda D, et al. Two- and threedimensional transthoracic and transesophageal echocardiographic findings in epithelioid hemangioma involving
the mitral valve. Echocardiography. 2008;25(4):4435.
9. Suwanjutah T, Singh H, Plaisance BR, et al. Live/real
time three-dimensional transthoracic echocardiographic
findings in primary left atrial leiomyosarcoma. Echocardiography. 2008;25(3):3379.
19. van Beek EJ, Stolpen AH, Khanna G, et al. CT and MRI
of pericardial and cardiac neoplastic disease. Cancer
Imaging. 2007;7:1926.
20. Kirkpatrick JN, Wong T, Bednarz JE, et al. Differential
diagnosis of cardiac masses using contrast echocardiographic perfusion imaging. J Am Coll Cardiol.
2004;43(8):141219.
21. Mulvagh SL, Rakowski H, Vannan MA, et al. American
Society of Echocardiography. American Society of
Echocardiography Consensus Statement on the
Clinical Applications of Ultrasonic Contrast Agents in
Echocardiography. J Am Soc Echocardiogr. 2008;21(11):
1179201; quiz 1281.
22. Heath D. Pathology of cardiac tumors. Am J Cardiol.
1968;21(3):31527.
23. Devig PM, Clark TA, Aaron BL. Cardiac myxoma arising
from the inferior vena cava. Chest. 1980;78(5):7846.
24. Alfonso Penta De Peppo, Luigi Sommariva, et al. Cardiac
myxoma arising from the inferior vena cava. Tex Heart Inst
J. 1992;19(4):28890.
25. Ferrans VJ, Roberts WC. Structural features of cardiac
myxomas. Histology, histochemistry, and electron microscopy. Hum Pathol. 1973;4(1):11146.
26. Harvey WP. Clinical aspects of cardiac tumors. Am J
Cardiol. 1968;21(3):32843.
27. Greenwood WF. Profile of atrial myxoma. Am J Cardiol.
1968;21(3):36775.
28. Yuan SM. Mitral valve myxoma: clinical features, current
diagnostic approaches, and surgical management. Cardiol
J. 2012;19(1):1059.
29. McCarthy PM, Piehler JM, Schaff HV, et al. The significance
of multiple, recurrent, and complex cardiac myxomas.
J Thorac Cardiovasc Surg. 1986;91(3):38996.
30. Peters MN, Hall RJ, Cooley DA, et al. The clinical syndrome
of atrial myxoma. JAMA. 1974; 230(5):695701.
31. DePace NL, Soulen RL, Kotler MN, et al. Two dimensional
echocardiographic detection of intraatrial masses. Am J
Cardiol. 1981;48(5):95460.
32. Srivastava R, Hsiung MC, Fadel A, et al. Transesophageal
echocardiographic demonstration of biatrial myxoma.
Echocardiography. 2004;21(2):1878.
33. Jaleski TC. Myxoma of the Heart Valves: Report of a Case.
Am J Pathol. 1934;10(3):399406.
34. Khairnar P, Hsiung MC, Mishra S, et al. The ability of live
three-dimensional transesophageal echocardiography
to evaluate the attachment site of intracardiac tumors.
Echocardiography. 2011;28(9):10415.
35. Meller J, Teichholz LE, Pichard AD, et al. Left ventricular
myxoma: echocardiographic diagnosis and review of the
literature. Am J Med. 1977;63(5):81623.
36. Snyder SN, Smith DC, Lau FY, et al. Diagnostic features of
right ventricular myxoma. Am Heart J. 1976;91(2):2408.
37. Vasquez JC, Rosales E, Dueas R, et al. A large pediculated
myxoma of the left ventricle causing outflow obstruction
in a young man. J Am Soc Echocardiogr. 2007;20(12):1413.
e11413.e3.
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
1521
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Section 5: Ischemic Heart Disease, Cardiomyopathies, Pericardial Disorders, Tumors and Masses
1523
SECTION 6
Congenital Heart Disease
Chapters
Chapter 71 Fetal Cardiac Imaging
Chapter 72 M-Mode and Two-Dimensional Echocardiography
in Congenital Heart Disease
Chapter 73 Real time 3D Echocardiography for
Quantification of Ventricular Volumes, Mass and
Function in Children with Congenital and Acquired
Heart Diseases
1527
CHAPTER 71
Fetal Cardiac Imaging
Aarti H Bhat
Snapshot
Fetal Physiology
INTRODUCTION
The field of fetal cardiology has made significant advances
along with all aspects of prenatal evaluation over the last
three decades. Almost all structural fetal heart disease is
amenable to a detailed in utero diagnosis that can then
be used to develop plans for fetal as well as postnatal
management of a pregnancy. Fetal ultrasound and twodimensional imaging are central to this diagnostic process.
Improvements in ultrasound techniques as well as our
enhanced understanding of fetal cardiac anatomy and
physiology have facilitated and broadened the scope of
this specialty. The purpose of this chapter is to familiarize
the reader with the basic framework of fetal cardiology
using a basic description of echocardiographic anatomy.
Liberal use of illustrative labeled screen images as well as
movie clips (on-line version) are intended to orient the
reader as well as provide an example of the anatomical
defect or finding being discussed.
Disease
Case Studies
1528
FETAL PHYSIOLOGY
Fetal circulation appears uniquely adapted to maximize
efficiency and oxygen delivery. Some of the most
exhaustive information on fetal physiology was originally
derived from fetal sheep.26 Similar data from the human
fetus is incomplete. Oxygenated blood is delivered to
the fetus through the umbilical vein. This vein enters the
porta hepatis and gives several branches to the left lobe
of the liver, distal to these is the ductus venosus. The
umbilical veins then arches toward the right lobe of the
liver, is joined there by the portal vein, and gives branches
to the right lobe of the liver. The ductus venosus then
continues on and connects to the inferior vena cava, as
do the left hepatic veins. Flow from the ductus venosus is
preferentially directed by the Eustachian valve, across the
foramen ovale to the left atrium. Nearly the entire return
from the superior vena cava enters the right ventricle
through the tricuspid valve.2731 Right ventricular output
1529
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FUNDAMENTALS OF FETAL
CARDIAC IMAGING
Two-dimensional imaging is the principal imaging
modality for fetal heart disease detection. Basic ultrasound
principles apply to fetal imaging, although with many
additional levels of technical challenges as well as
opportunities. In general, a combination of obstetric and
pediatric cardiology skills is required. Flexibility, familiarity
as well as perseverance are essential to get as complete
and accurate a fetal cardiac examination as possible. The
small size of fetal cardiac structures as well as the relatively
faster fetal heart rate can challenge the spatial as well as
temporal resolution capabilities of the ultrasound system.
Accordingly, a higher frequency transducer, minimal
distance between the fetal heart and the transducer on
the maternal abdomen, appropriate depth and width
of the region of interest, and dynamic focusing are all
important considerations and need to be optimized. Since
axial resolution typically exceeds radial resolution, the
transducer can be moved on the maternal abdomen so as
to insonate the region of interest at minimal angulation.
Similar to the pediatric cardiac exam, each region of
interest needs to be seen in multiple views to get an accurate
impression. Again, similar to a pediatric cardiac exam,
a segmental completeness is necessary. Fetal position,
movement, and variable transducer positions make this a
literal moving target and while a protocolized progression
of sweeps and views cannot be realistically applied,
every attempt should be made to demonstrate as many
anatomical and functional components of each segment.
A lower frequency transducer, even a nonobstetric one
may be occasionally helpful in a later trimester gestation,
where more penetration is required. This strategy may also
be required in the rare event of an aliasing Doppler signal
with the usual obstetric transducer. Harmonic imaging
may enhance the bloodtissue interface and permit
better assessment in some challenging cases. In early
1531
Four-Chamber View
This view is part of the obstetric standard for anatomy
scan. Some very important and critical cardiac lesions are
readily picked up in this view such as hypoplastic left or
right heart, mitral or tricuspid atresia, atrioventricular (AV)
canal defects balanced or unbalanced, double inlet left
ventricle, single ventricle type situations, and large septal
defects. While such a diagnosis may be striking at the very
onset of the examination, this view alone is inadequate
for a complete assessment. In more subtle situations of
ventricular or atrioventricular valve discrepancy, each of
these structures needs to be measured in the cardiac cycle
that reaches its maximal dimension and compared to
gestational age-based norms.5860 This view demonstrates
1532
Fig. 71.3: Inferior vena cava (IVC), hepatic veins (Hep V), and
ductus venosus (DV). The inferior vena cava is right-sided in this
fetus with levocardia. It is joined by the right and left hepatic veins
as well as the ductus venosus, which can be identified as a turbulent region on color Doppler along the course of the umbilical vein
to the IVC. These venous structures connect to the right atrium
(RA). This Figure correlates with Movie clip 71.3.
Fig. 71.5: Color Doppler across interatrial membrane. The interatrial membrane region is marked * and indicates right-to-left shunting from the right atrium (RA) to the left atrium (LA) in this normal
fetus. (Movie clip 71.5).
1533
Fig. 71.6: There is laminar color Doppler flow across the tricuspid
(TV) and mitral (MV) valves from the right (RA) and left atria (LA)
to their respective ventricles (RV, LV). There is no obvious shunting at the interventricular septal level. (Movie clip 71.6).
Fig. 71.7: Pulsed wave Doppler across the tricuspid valve showing
a wave dominance in this 28-week fetus. (Movie clip 71.7).
Fig. 71.8: Pulsed wave Doppler across the mitral valve showing in
this 28-week fetus. (Movie clip 71.8).
Fig. 71.10: Pulse Doppler of pulmonary vein. In this image, the right
upper pulmonary vein is being interrogated after it was identified on
color Doppler. Note that there is some contamination of the signal
with the right pulmonary artery signalseen as the sharp systolic
wave below the baseline. This coincidentally demonstrated the swave of the pulmonary venous Doppler, followed by the d-wave.
(Movie clip 71.10).
1534
Fig. 71.11: Short-axis mitral valve, interventricular septum, moderator band attachment. The left ventricle (LV) is identified by its
circular contour, presence of two papillary muscles and mitral valve
(MV), and smooth interventricular septum. The right ventricle is
the anterior chamber and its outflow is seen wrapping around the
left ventricle in a relatively oblong manner. The septal attachment
of the moderator band is identified. (Movie clip 71.11).
1535
Fig. 71.15: Two-dimensional (2D) right ventricular outflow, pulmonary valve (PV). The right ventricle (RV) is the anterior chamber
and its outflow is seen wrapping around the left ventricle in a relatively oblong manner. The PV and main pulmonary artery (MPA)
are seen in this image. The aortic valve is seen at the crux of the
heart at the same level as the PV and is marked as X in this image.
This image correlates with Movie clip 71.15.
and the bulging of thin foraminal rims into the left atrium
indicates the direction of shunting at this level from right
atrium to left atriumthis can be confirmed on color
Doppler in orthogonal views of the interatrial membrane.
The tricuspid valve and the right ventricle are mildly
dominant toward latter gestation, although both sides
appear relatively symmetric in earlier gestation. Defects
1536
Fig. 71.21: Short axis of aortic valve (marked as X). The symmetric and trileaflet anatomy of the normal aortic valve is seen in this
image. This view is particularly useful to illustrate the morphology
of an abnormal aortic valve as annular hypoplasia, valve thickening, and asymmetry. The tricuspid valve (TV) leading to the right
ventricle and right ventricular outflow tract (RVOT) is noted. The
patent foramen ovale (PFO) is well profiled in this view. (Movie
clip 71.21).
1537
Fig. 71.23: Two-dimensional (2D) of ductal and aortic arches, isthmus (*). The ductal arch (DuAr) is anterior and describes a gradual
curve slightly rightward and posteriorly where it is met at the isthmus (*) by the more posterior and acutely angulated aortic arch
(AoAr). (Movie clip 71.23).
Fig. 71.24: Transverse aortic arch, and head and neck vessels.
The fetal head is to the left of this screen image. The transverse
aorta (TrAo) has been opened out to demonstrate each of the
head and neck vessels, the last one being the left subclavian
artery (LSCA). This image set is important to demonstrate head
and neck vessels as well as indicators or aortic arch anomalies.
The aortic isthmus is distal to the LSCA and needs to be clearly
visualized to rule out aortic coarctation. (Movie clip 71.24).
Fig. 71.25: Two-dimensional (2D) of three-vessel view and pulmonary artery (PA) bifurcation. The orientation of the fetal chest
is marked. The fetal spine (Sp) marks the posterior aspect. The
descending aorta (DAo) is seen just to the left and anterior of the
spine. The three vessels noted from left to right as well as anterior
to posterior are the PA, aorta (Ao), and right-sided superior vena
cava (RSVC) in that order of location as well as decreasing size.
Identification of the location as well as relative sizes of the great
vessels in this view is critical in detecting and confirming presence
of obstruction, size discrepancy as well as arch anomalies. The
right pulmonary artery (RPA) is seen to arise from the main PA and
traverses posterior to the aorta as well as RSVC as it heads to the
right lung hilum. (Movie clip 71.25).
1538
Fig. 71.28: Two-dimensional (2D) three-vessel view. In this additional example of a normal three-vessel view, the orientation of
the fetal chest is marked. The fetal spine (Sp) marks the posterior
aspect. The descending aorta (DAo) is seen just to the left and
anterior of the spine. The three vessels noted from left to right as
well as anterior to posterior are the pulmonary artery (PA), aorta
(Ao), and right-sided superior vena cava (RSVC) in that order of
location as well as decreasing size. Identification of the location
as well as relative sizes of the great vessels in this view is critical
in detecting and confirming presence of obstruction, size discrepancy as well as arch anomalies. The right pulmonary artery (RPA)
is seen to arise from the main PA and traverses posterior to the
aorta as well as RSVC as it heads to the right lung hilum. (Movie
clip 71.28).
Fig. 71.35: Umbilical cord vessels. The umbilical vein (*) is relatively thin walled and of larger caliber. There are two umbilical
arteries (-) that are thicker walled and of smaller lumen.
1539
1540
1541
vessels from the top of the curve. The ductal arch describes
a tighter curve and is described as a hockey stick as it
joins the aortic arch. Sweeping back and forth amidst
these two arches allows separation of these arches. A
proportionate size, as well as laminar antegrade flow on
color Doppler are reassuring for preserved fetal physiology
and right-to-left shunting at the ductal level. Resistance
and pulsatility indices can be calculated and are valuable
indicators to differentiate increased flow across the
ductus (increased right ventricular output) from ductal
constriction (due to maternal nonsteroidal ingestion).70,71
A size discrepancy needs further assessment. In ductaldependent systemic circulations, there is antegrade flow in
the ductal arch, possible abnormal flow in the isthmus, and
potentially retrograde flow in the transverse aortic arch. In
ductal-dependent pulmonary circulation, the ductus is
abnormally angulated and arises more vertically from
the undersurface of the aortic arch, and has retrograde
flow indicating that even in the fetal stage, pulmonary
circulation is dependent on a retrograde ductus. Identifying
both these entities is critical as it has ramifications on fetal
counseling, preparing a more detailed birth plan including
initiation of prostaglandins soon after birth.
1542
1543
Case VSD 1. Fig. 71.40: Color Doppler ventricular long axis with
ventricular septal dropout in high muscular region confirms the
presence of a septal defect in this region. (Movie clip 71.40).
1544
Case VSD 2. Fig. 71.44: Orthogonal view of midmuscular ventricular septal defect (VSD). This is the same patient and imaging
session as in Figure 71.1. In this image, the previously suspected
ventricular septal defect is confirmed on color Doppler as well as a
nearly perpendicular angle of insonation.
Case AVCD. Fig. 71.45: Four-chamber identification of atrioventricular canal components. A fetal echocardiogram was performed
for this 38-year-old G6P4 with Marfan syndrome and a fetus at 28
5/7 weeks gestation with trisomy 21 confirmed on amniocentesis.
The left ventricle is left-sided and on the left of the screen. The
right ventricle is identified with its apical trabeculations. A large
atrial septal defect including the septum primum (**) is seen along
with a large ventricular septal defect (*). The common atrioventricular valve is seen to sit astride this large defect in the crux of
the heart.
Case AVCD. Fig. 71.47: Short-axis view of common atrioventricular (AV) valve demonstrates the single valve, and its anterior and
posterior leaflets as well as right and left components. The anterior
leaflet has connections to the crest of the interventricular septum
in this example, indicating that it is likely to be Rastelli type 1. The
pulmonary valve (PV) is noted anteriorly and is of a good size.
1545
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1547
Case Dextrocardia. Fig. 71.55: Smaller pulmonary artery and bifurcation. The right pulmonary artery (RPA) is red on color Doppler
as it moves toward the transducer; the left pulmonary artery (LPA)
is blue as it moves away. The main pulmonary artery (MPA) is of
smaller caliber than the aorta (Ao) that is directed leftward from its
anterior position. (Movie clip 71.55).
as Doppler applications assessing tissue motion and realtime 3D imaging continue to develop, bioeffects on the
fetus will need to continue to be monitored. As there are
no strictly defined limits established, use of ultrasound
energy in fetal echocardiography is best expressed by the
ALARA principleas low as reasonably achievable.108
1548
Case TA. Fig. 71.58: Four-chamber asymmetry. This four-chamber equivalent view demonstrates asymmetry in ventricular chamber size, the right-sided right ventricle (RV) is smaller, and its apex
does not reach up to the cardiac apex. The tricuspid valve above
it seems to have plate-like atresia between the right atrium (RA)
and RV. A large ventricular septal defect (*) is seen separating
the smaller right ventricle from the larger and apex-forming left
ventricle (LV). The mitral valve (MV) is seen in its open diastolic
position. The interatrial foraminal membrane is seen bulging into
the left atrium (LA), suggesting that there is right-to-left flow at this
level. (Movie clip 71.58).
Case TA. Fig. 71.59: d-malposition of the aorta. The aorta (Ao)
is identified due to its relationship with head and neck vessels.
This vessel arises from the anterior and rightward bulboventricular chamber (BVC). Blood enters the BVC through a currently
unrestrictive ventricular septal defect (VSD) from the left ventricle
(LV). There is no aortomitral continuity and the aorta is of good
size. (Movie clip 71.59).
Case TA. Fig. 71.61: Origin of malposed great arteries, subpulmonic ventricular septal defect (VSD). The aorta (Ao) is anterior,
smaller, and arises from the anterior diminutive right ventricle
(RV). The pulmonary artery (P) arises posteriorly from the larger
and dominant left ventricle (LV). This indicates ventriculoarterial
discordance. A subpulmonic VSD (*) and its relationship to the
great arteries is seen clearly. Note that the subpulmonic conus
is minimally thickened and deviated into the subpulmonic region.
(Movie clip 71.61).
1549
Case TA. Fig. 71.62: Abnormal 3VV in DTGA. The fetal spine (Sp)
and anterior aspects are marked. The aorta is rightward and anterior to the pulmonary artery (d-malposed). The right pulmonary
artery (RPA) origin from the pulmonary artery (PA) is seen before
this vessel continues as the ductal arch (DuAr) to the descending
aorta (DAo). (Movie clip 71.62).
Case TA. Fig. 71.63: DTGA, anterior aorta bulboventricular chamber. The reader is encouraged to use the previous images to determine the anatomy in this labeled image. (Movie clip 71.63).
Case HLHS. Fig. 71.65: Bicaval view showing foraminal flap. The
superior (SVC) and inferior (IVC) vena cavae are seen to enter
the right atrium in this bicaval view. The foraminal membrane is
seen with the crest of the flap directed toward the right atrium (*),
indicating left-to-right flow at this level. Normal atrial level shunting
in the fetus is always from the right to the left and presence of an
opposite direction of flow indicated impaired left atrial drainage
from anatomical or functional causes. (Movie clip 71.65).
Three-Dimensional Imaging
3D and 4D (3D + fourth dimension of time) fetal echocardiography requires acquisition of a volume of imaging
data. Just as two-dimensional data is defined as a pixel in
1550
Case HLHS. Fig. 71.69: Color Doppler showing retrograde transverse aortic arch flow. This image is derived by rotation of the
transducer from the position that demonstrated Figure 71.5. Here
the aortic arch is identified by the head and neck vessels, and the
transverse aorta (AoAr) is seen to fill retrograde from the isthmus
and the antegrade ductal arch (DuAr). (Movie clip 71.69).
1551
Case HLHS 2. Fig. 71.72: Four-chamber viewsmaller left ventricle (LV) and mitral atresia. In this case, a large and hypertrabeculated right ventricle (RV) contrasts with the smaller, non-apexforming LV. The smaller left atrium (LA) is separated from the
larger right atrium (RA) by a slightly thickened interatrial septum
with a central defect; its edges are bowing into the right atrium.
(Movie clip 71.72).
1552
Case HLHS 2. Fig. 71.74: Color flow across large apical ventricular septal defect (VSD). This is the only source of blood flow into
the left ventricle from the right ventricle. (Movie clip 71.74).
1553
Case PA. Fig. 71.80: Asymmetric four chamber showing tricuspid and right heart hypoplasia. The tricuspid annulus is small and
appears to be dysplastic. The right ventricle is smaller and non
apex-forming. (Movie clip 71.80).
Case PA. Fig. 71.81: Longitudinal view showing aortic outflow and
ventricular septal defect (VSD; *). The left atrium (LA) and left ventricle (LV) are noted here, with presence of aortomitral continuity.
The aortic outflow (Ao) is astride a ventricular septal defect (*).
(Movie clip 71.81).
1554
Case PA. Fig. 71.82: Retrograde ductal arch (DuAr) in fetus with
pulmonary atresia. (Moive clip 71.82).
Case PR. Fig. 71.85: Color Doppler right ventricular outflow showing free pulmonary incompetence and ductal reversal. A wide
regurgitant jet is seen to enter the right ventricle (RV) from across
the plane of the previously noted pulmonary annulus. In fact, this
reversal is seen to extend into and include the main pulmonary artery (MPA) and the ductal arch (DuAr). (Movie clip 71.85).
1555
Case PR. Fig. 71.86: 3VV dilated pulmonary valve and branch
pulmonary artery (PA). (Movie clip 71.86).
Case PR. Fig. 71.87: Color Doppler both arches flow reversal in
ductal arch.
Case PR. Fig. 71.88: Pulse Doppler in ductal arch showing diastolic reversal or tofro flow.
Case PR. Fig. 71.89: Pulse Doppler across ductus venosus showing return to baseline.
Case PR. Fig. 71.91: Pulse Doppler of umbilical vein with no obvious or significant venous notching.
1556
CASE STUDIES
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
Case
Case
Case
Case
Case
Case
Case
Case
Case
Case
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assessment of fetal cardiac function. Ultrasound Obstet
Gynecol. 1992;2(6):43445.
78. Carceller-Blanchard AM, Fouron JC. Determinants of the
Doppler flow velocity profile through the mitral valve of
the human fetus. Br Heart J. 1993;70(5):45760.
80. Tulzer G, Khowsathit P, Gudmundsson S, et al. Diastolic
function of the fetal heart during second and third trimester:
a prospective longitudinal Doppler-echocardiographic
study. Eur J Pediatr. 1994;153(3):1514.
81. Mkikallio K, Vuolteenaho O, Jouppila P, et al. Ultrasonographic and biochemical markers of human fetal cardiac
dysfunction in placental insufficiency. Circulation. 2002;
105(17):205863.
82. Hernandez-Andrade E, Figueroa-Diesel H, Kottman C,
et al. Gestational-age-adjusted reference values for the
modified myocardial performance index for evaluation
of fetal left cardiac function. Ultrasound Obstet Gynecol.
2007;29(3):3215.
83. Friedman D, Buyon J, Kim M, et al. Fetal cardiac function
assessed by Doppler myocardial performance index
(Tei Index). Ultrasound Obstet Gynecol. 2003; 21(1):336.
84. Meriki N, Welsh AW. Technical considerations for measurement of the fetal left modified myocardial performance
index. Fetal Diagn Ther. 2012;31(1):7680.
85. Van Mieghem T, Gucciardo L, Lewi P, et al. Validation of
the fetal myocardial performance index in the second and
third trimesters of gestation. Ultrasound Obstet Gynecol.
2009;33(1):5863.
86. Donofrio MT, Bremer YA, Schieken RM, et al. Autoregulation of cerebral blood flow in fetuses with congenital
heart disease: the brain sparing effect. Pediatr Cardiol.
2003;24(5):43643.
87. Clark JM, Case CL. Fetal arrhythmias. In: Gillete PC,
Garson A, editors. Clinical Pediatric Arrhythmias. 2nd ed.
Philadelphia: WB Saunders; 1999:293302.
88. Matias A, Montenegro N, Areias JC, et al. Haemodynamic
evaluation of the first trimester fetus with special emphasis
on venous return. Hum Reprod Update. 2000;6(2):17789.
89. Wheeler T, Murrills A. Patterns of fetal heart rate during
normal pregnancy. Br J Obstet Gynaecol. 1978;85(1):1827.
90. Carvalho JS, Prefumo F, Ciardelli V, et al. Evaluation of fetal
arrhythmias from simultaneous pulsed wave Doppler in
pulmonary artery and vein. Heart. 2007;93(11):144853.
91. Fouron JC. Fetal arrhythmias: the Saint-Justine hospital
experience. Prenat Diagn. 2004;24(13):106880.
92. Kleinman CS, Nehgme RA. Cardiac arrhythmias in the
human fetus. Pediatr Cardiol. 2004;25(3):23451.
93. Southall DP, Richards J, Hardwick RA, et al. Prospective
study of fetal heart rate and rhythm patterns. Arch Dis
Child. 1980;55(7):50611.
94. Krapp M, Kohl T, Simpson JM, et al. Review of diagnosis,
treatment, and outcome of fetal atrial flutter compared
with supraventricular tachycardia. Heart. 2003;89(8):
91317.
95. Krapp M, Baschat AA, Gembruch U, Geipel A, Germer U.
Flecainide in the intrauterine treatment of fetal supraventricular tachycardia. Ultrasound Obstet Gynecol.
2002;19(2):15864.
96. Oudijk MA, Ruskamp JM, Ververs FF, et al. Treatment of fetal
tachycardia with sotalol: transplacental pharmacokinetics
and pharmacodynamics. J Am Coll Cardiol. 2003;42(4):
76570.
97. Merriman JB, Gonzalez JM, Rychik J, et al. Can digoxin
and sotalol therapy for fetal supraventricular tachycardia
and hydrops be successful? A case report. J Reprod Med.
2008;53(5):3579.
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CHAPTER 72
M-Mode and Two-Dimensional
Echocardiography in Congenital
Heart Disease
Neeraj Awasthy, Savitri Shrivastava
Snapshot
Paent Preparaon
Imaging
Dextrocardia
Aortopulmonary Window
Gerbode Defect
Aorc Regurgitaon
Aortocameral Communicaons
Aorc Override
Truncus Arteriosus
Coronary Aneurysms
Aorc Aneurysm
Tricuspid Atresia
Heterotaxy Syndrome
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PATIENT PREPARATION14
Prior to taking the patient for an echocardiographic
study, all the available clinical data should be carefully
perused and the oxygen saturation checked. This helps in
total evaluation of lesions. Pediatric echocardiographic
examination should be performed when the child is quiet
and cooperative or else one may miss certain important
observations. In a crying baby alter intrathoracic pressures
resulting in fallacious gradients and other vital findings.
Most of the neonates and infants can be quietened
by a feed, handling by an experienced nurse, using a
pacifier, and keeping them adequately warm. Wrapping
a small child is in itself not only a proper positioning
method for small children and infants but a cuddled
up wrapped infant can be easily quietened for a proper
echocardiographic examination (Fig. 72.1). Certain
children can also remain quiet by familiarizing them with
the surroundings and a friendly environment with some
Fig. 72.1: The steps for appropriately wrapping up an infant for proper cardiac evaluation. Cuddled up neonate is well suited and cooperative for any echocardiographic examination. Step1: Laying the sheet with single fold on a flat surface (bed); Step 2: Demonstrates
the end, which is picked up and wrapped on the babys opposite feet; Step 3: Demonstrates step 2 on the baby; Step 4: Wrapping the
left over upper end of the sheet on the upper limb; Step 5: Repeating the step of wrapping the upper arm for the other arm; Step 6: Child
with restrained both upper limbs; Step 7 and Step 8: The lower part of the sheet is wrapped around the lower limbs to cuddle up the child.
Subcostal Window7
Evaluation of congenital heart lesions generally starts with
the subcostal view. This provides information regarding
the situs, which is a crucial step in the diagnosis of CHD.
Imaging the inferior vena cava (IVC) and abdominal aorta
in cross section gives this information (Fig. 72.2). In small
infants if the transducer is pressed too deep, impairment
of respiration may occur. Intensive pressure may also
compress on the IVC impairing the venous return.
At times, it may lead to nonvisualization of an otherwise
patent IVC. The pulsations, color flow mapping, and pulse
Doppler interrogation identifies the IVC and the aorta.
Table 72.1: Conventional Views of Echocardiography
IMAGING
The anatomical reference for the echocardiographic
planes is the major axis of the heart and not the major
axis of the body. Displaying images in an anatomically
correct fashion allows better understanding of anatomy,
particularly in cases of complex CHD. For an anatomically
correct display, the apex of the imaging sector is placed at
the bottom of the screen in the subcostal and the apical
views, and for the rest of the views it is placed at the top of
the screen. The aim of the study is to image all structures
of the heart and great vessels in all possible planes so that
one can get a three-dimensional image from the twodimensional (2D) study. The study should be performed
in a systematic sequence in all patients so as to minimize
errors in diagnosis. In each plane, the transducer should
be swept across the heart and great vessels in a specific
direction with and without color flow imaging (CFI). Using
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Subcostal
Sagittal (B1 caval)
Coronal
Paracoronal view
Apical
4C view
5C view with aorta
5C view with pulmonary artery
Parasternal
Parasternal long-axis view
Psax ( parasternal short-axis view)
High parasternal short-axis view (ductal view)
Suprasternal windows
Long-axis view
Short-axis view
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Fig. 72.2: Two-dimensional echocardiography. Subcostal shortaxis at the level of abdominal vessels. Inferior vena cava and aorta
are shown in cross section with aorta (red) to left of spine and
inferior vena cava (blue) to right of spine (*).
D1
Figs 72.3A to E
D2
E1
E2
F1
F2
F3
F4
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Figs 72.3A to F: Two-dimensional echocardiography subcostal window. (A) Subcostal coronal (four-chamber equivalent) view showing
interatrial septum, connection of right upper pulmonary vein (RUPV) to left atrium. Descending aorta ( ) in short axis is imaged behind
the left atrium; (B) Subcostal coronal view with posterior-to-anterior sweep showing left ventricular outflow tract and aorta with color flow
mapping (blue); (C) Far anterior tilting shows right ventricular outflow tract; (D1 and D2) Subcostal paracoronal view with right anterior
tilt showing right ventricle (RV) and right ventricular outflow tract. (E1 and E2) Subcostal paracoronal view with color compare showing
right ventricular outflow (*) tract, main pulmonary artery, and right pulmonary artery; (F1 to F4) Subcostal sagittal view of the muscular
septum with right-to-left sweep. This view demonstrates the short axis of the left ventricle (LV) at the level of the mitral valve with sweep
up to the apex of the heart. The color comparison at the scale of 3536 is especially important to look for a small muscular ventricular
septal defect (VSD), especially in the setting of the increased pulmonary artery pressures (not shown in the illustration).
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Figs 72.4A to D: Two-dimensional transthoracic echocardiography. (A) Apical four-chamber view in posterior plane showing coronary
sinus (CS) coursing from left to right in atrioventricular groove; (B) Apical four-chamber view showing all four cardiac chambers, the
interatrial septum, and interventricular septum. Offsetting of atrioventricular valves (attachment of tricuspid valve more toward apex than
mitral valvearrow) is seen; (C) Apical four-chamber view in anterior plane (apical five-chamber view) showing left ventricular outflow
tract and aorta; (D) Same view in far anterior tilt visualizes right ventricular outflow tract and main pulmonary artery. (Ao: Aorta; CS:
Coronary sinus; LA: Left atrium; LV: Left ventricle; MPA: Main pulmonary artery; RA: Right atrium; RV: Right ventricle).
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Figs 72.5A to D: Two-dimensional transthoracic echocardiography. (A) Parasternal long-axis view of left ventricle showing the left
ventricular inflow and outflow tract with anteriorly placed RV cavity; (B) Parasternal long-axis view with anterior tilt showing right
ventricular outflow tract. (C and D) Parasternal long axis with posterior tilt and color compare showing right ventricular inflow. (Ao: Aorta;
LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right atrium; RVOT: Right ventricular outflow tract).
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Figs 72.6A to D: Two-dimensional transthoracic echocardiography. (A) Parasternal short-axis view at the base of the heart showing
aorta (Ao), right ventricular outflow tract, pulmonary valve, main pulmonary artery, bifurcation of main pulmonary artery, and right and
left pulmonary arteries; (B) Parasternal short-axis view of left ventricle at the level of the mitral valve showing the anterior mitral leaflet
(arrow) and the posterior mitral leaflet (arrow); (C) Parasternal short-axis view of left ventricle at the level of papillary muscle showing
the location of papillary muscles, anterolateral at 4 oclock and posteriomedial at 8 oclock, trabecular part of interventricular septum,
and right ventricular cavity; (D) Parasternal short-axis view toward apical part of ventricular septum. (LPA: Left pulmonary artery; LV: Left
ventricle; MPA: Main pulmonary artery; RPA: Right pulmonary artery; RV: Right ventricle).
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High Parasternal or
Ductal View (Fig. 72.8)
The classic short-axis view visualizing the aorta and PA is
initially obtained from just below the clavicle, a level higher
than the usual parasternal views. From this location, the
transducer is rotated anticlockwise. During this process,
the right PA gradually goes away from view. The main PA
is then seen continuing as the left PA. The descending
thoracic aorta then is in view. The duct is located at the
junction of left PA with the descending aorta. This is called
ductal view.
Fig. 72.7: Two-dimensional echocardiography. Parasternal shortaxis view showing origin of both the right and left coronary arteries.
(LCA: Left coronary artery; RCA: Right coronary artery; Ao: Aorta).
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Fig. 72.10: Two-dimensional echocardiography with color flow compare. Suprasternal short-axis view showing aorta in short axis, right
pulmonary artery in long axis and innominate vein joining superior vena cava, all four pulmonary veins joining left atrium. (LA: Left
atrium; RPA: Right pulmonary artery; SVC: Superior vena cava; AO: aorta; Inn V: Innominate vein).
DEXTROCARDIA
For patients with dextrocardia, attempts should be made
to perform the same views keeping the transducer on
the right side of the chest (Fig. 72.11). The marker on the
transducer should point in the same overall direction as
for patients without dextrocardia. This enables consistency
in image orientation and display.
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Chest wall deformities: These are common in patients with congenital heart disease. Thus, pectus excavatum or carinatum produces
poor parasternal images. Also, scoliosis causes the heart to shift to the ipsilateral side and produces difficulty in imaging
Diaphragmatic hernia: In this condition, the bowel loops can come in front of the chest wall. Since air is a poor conductor of
ultrasound, it can cause unexplained difficulties in imaging. The plain X-ray of the chest easily diagnoses the condition
The postoperative state: Chest wall edema, bandages, and chest wall drains in the immediate postoperative period and
deformity of the sternum in the late postoperative state frequently cause problems in imaging. This is especially true for midline
structures. Following the arterial switch and the LeCompte maneuver, the pulmonary artery frequently lies behind the sternum.
Similarly, midline structures like conduits may be poorly visualized.
Grown up congenital heart disease: It is frequently difficult to get good images in a grown up patient with congenital heart disease.
This is especially true for structures like the pulmonary artery, pulmonary and systemic veins, descending thoracic aorta, ductal
area, etc. even with use of adult transducer. Associated with the poor window is the inability to get good color flow images or
continuous wave Doppler envelopes
Figs 72.12A and B: Nonoptimal scales can result in overestimation of tricuspid valve regurgitation. (A) Apical four-chamber view
showing tricuspid valve regurgitation with splaying of the colors (at color scale of 32); (B) Apical four-chamber view in the same patient
showing tricuspid valve regurgitation with no splaying of the colors at color scale of 72.
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Figs 72.13A and B: Optimal color gain settings are essential to prevent graining in the field of imaging. (A) Four-chamber (inverted
view) showing graining in the field of interest with gain settings of 90%; (B) The same patient with trace regurgitation and minimal graining after optimizing color gains.
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Figs 72.14A and B: (A) Doppler signal with inappropriate gain setting resulting in falsely increased gradients. Note excessive splaying
with a gradient of 85 mm Hg; (B) The same patient with optimized gains with a gradient of 51 mm Hg (+).
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Figs 72.15A and B: (A) High frequency signal not getting aligned in view of the pulsed wave Doppler exceeding the Nyquist limit;
(B) The same patient by appropriately increasing the scale of the measurements, and changing from pulsed wave to continuous wave
Doppler.
PRINCIPLES OF SEQUENTIAL
CHAMBER ANALYSIS913
The cardinal principle of sequential chamber analysis states
that the morphology of a chamber should be determined
on the basis of its most constant component. Thus, one
should not use a component to identify a structure if
that component itself is variable. The evaluation of any
congenital heart involves a systemic sequential analysis
that involves the following steps:
Identification of abdominal situs.
Identification of atria and atrial arrangement.
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Fig. 72.18: Two-dimensional echocardiography. Subcostal shortaxis view at the level of abdominal vessels. Inferior vena cava and
aorta are shown in cross-section with aorta (red) and inferior vena
cava (blue) to right of spine. Spine (*). (Ao: Aorta; V: IVC).
Figs 72.19A and B: Two-dimensional echocardiography in subcostal coronal view with superior tilt showing (A) broad-based right atrial
appendage (RAA); (B) narrow, finger-like left atrial appendage (LAA).
Identification of Atrium
The atrial appendage: It is the most constant feature for
atrial identification. The right atrial appendage is a broad
triangular structure with a broad junction with the rest
of the atrium (Figs 72.19A and B). It has coarse pectinate
muscle within the appendage, which extend all around
the vestibule of the appendage and atrial junction. The
morphological left atrial appendage is tubular with a
narrow junction with the rest of the atrium (Fig. 72.20).
The posterior wall is smooth, and the pectinate muscle is
confined only to the anterior quadrants of the vestibule.
Echocardiographically, the shape of atrial appendages is
best visualized by a combination of the subcostal coronal
view (right and left atrial appendages) and parasternal
short axis at great vessel level (left atrial appendage). The
pectinate muscles, however, cannot be well identified by
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Echocardiographic Identification of
Isomerism
Right and left isomerism should be strongly suspected on
echocardiography when the following findings are present.
Right Isomerism: Also called the asplenia syndrome, this
is strongly suspected on the abdominal scan of the great
vessels. The aorta and IVC lie on the same side of the
spine (left or right) instead of on either side. The aorta
lies posterior to the IVC. The IVC opens into either the
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The offsetting sign and moderator band are best identified by the apical four-chamber view
The chordal attachment of the morphological tricuspid valve to the interventricular septum is best seen in the subcostal
coronal views
The two discrete papillary muscles of left ventricle and the apical trabeculations of right ventricle are best appreciated in parasternal
short-axis views. The transducer is focused inferiorly starting at the base of the heart. The papillary muscles are first seen followed
by the apical trabeculations
Absence of an atrioventricular valve, common atrioventricular valve, and straddling are best seen in the apical views. The common
atrioventricular orifice is also well appreciated in the subcostal sagittal views
Fig. 72.21: Two-dimensional echocardiography. Apical four-chamber view showing all four cardiac chambers and offsetting of atrioventricular valves (attachment of tricuspid valve more toward apex
than mitral valvearrow). (LA: Left atrium; LV: Left ventricle; RA:
Right atrium; RV: Right ventricle).
Fig. 72.22: Two-dimensional echocardiography. Apical fourchamber view showing discordant atrioventricular connection, right
atrium to left ventricle, and left atrium to right ventricle. Moderator
band on left side (anatomical right ventricle) and reverse offsetting
of atrioventricular valves are seen (arrow).
Figs 72.23A and B: Two-dimensional echocardiography. Apical four-chamber view showing univentricular atrioventricular (AV)
connection. (A) Tricuspid atresia (arrow), hypoplastic right ventricle; (B) Mitral atresia (arrow), with hypoplastic left ventricle (LV).
(LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Echocardiographic Identification of
Ventricular Morphology
The offsetting sign of atrioventricular valves: This sign,
if present, forms the single most important feature in
identification of ventricular morphology. It takes into
account that the AV valve always follows its ventricle.
The tricuspid valve is attached more apically compared
to the mitral valve. Thus, a ventricle which has its AV
valves attached more apically will be the morphological
RV. The disadvantage with this sign is that when a VSD
extends to the inlet septum or in univentricular hearts, this
offsetting sign is lost and therefore cannot be used as a
feature for ventricular morphological identification.
The ventricular septal and apical trabeculation: The
morphologists consider this as the most constant feature of
ventricular morphology. The RV apical trabeculations are
coarse. The moderator band contributes to this coarseness.
The RV side of the septum is seen to be coarser compared
to the LV side of the septum. Echocardiographically, this
feature becomes difficult to identify if one of the ventricles
is severely hypoplastic or there is a true single ventricle.
Attachment of the chordae and papillary muscle to the
septum: The RV side of the septum gains attachment of the
chordae and papillary muscle. The left ventricular side of
the septum is always free of chordal attachment.
Papillary muscles: The LV has two discrete papillary
muscles. The RV has three or more papillary muscles.
Echocardiographic Identification of
Atrioventricular Connection
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Ventriculoarterial Connection
The ventriculoarterial connections can be divided into the
following types:
Concordant ventriculoarterial connection.
Discordant ventriculoarterial connection.
Double outlet ventricle (right or left).
Single outletpulmonary atresia, aortic atresia.
Common outlet.
Fig. 72.24: Two-dimensional echocardiography. Parasternal longaxis view showing ventriculoarterial discordance. (AO: Aorta; LV:
Left ventricle; PA: Pulmonary artery; RV: Right ventricle).
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Fig. 72.25: Two-dimensional echocardiography. Apical four-chamber view with anterior tilt showing double outlet right ventricle with
nonrestrictive inlet ventricular septal defect (VSD; *). (AO: Aorta;
LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).
Figs 72.27A and B: A case of ventricular septal defect and pulmonary atresia. Subcostal paracoronal view with color compare showing
the atretic right ventricular outflow tract (arrow) and ventricular septal defect (*). (AO: Aorta; PA: Pulmonary artery.
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Figs 72.28A and B: Two-dimensional echocardiography of an infant with truncus arteriosus. (A) Subcostal coronal view with anterior
tilt showing the single outflow-truncus arising from the ventricles; (B) Parasternal short-axis view showing the pulmonary arteries arising
from the common trunk and bifurcating into left and right pulmonary arteries. (LPA: Left pulmonary artery; LV: Left ventricle; MPA: Main
pulmonary artery; RPA: Right pulmonary artery; T: Truncus).
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Increase in RV pressures may be because of infundibular or valvular pulmonary stenosis, obstruction in branch pulmonary arteries, or obstruction
in pulmonary vascular bed (as in elevated pulmonary vascular resistance).
Increase in pressures in RV will also be seen in large
VSD; similarly, a large PDA would lead to significant
increase in PA pressures due to direct transmission
of pressures.
The magnitude of the gradient from a chamber outflow
would also be dependent on the magnitude of shunt
into the chamber (Fig. 72.29).
This may lead to exaggerated gradients even in
hemodynamically insignificant lesions, namely
exaggerated pulmonary gradients in pre-tricuspid
shunts (like ASD), exaggerated mitral and aortic
valve gradients in post-tricuspid shunts like VSD or
PDA.
The secondary manifestations of the shunt lesions may
themselves lead to exaggerated secondary effects, for
example, dilatation of LV leading to the mitral annular
dilatation on account of post-tricuspid shunt may lead
to mitral regurgitation and this may further lead to
mitral annular dilatation and LV dilatation.
Since a shunt lesion is a communication between two
chambers, the gradient between the two chambers will
be dependent on the size of the defect (Figs 72.31A and B).
The size of the defect in two dimensions may be a
useful guide in deciding the degree of shunt.
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Figs 72.31A and B: (A) Continuous wave Doppler signal of a patent ductus arteriosus, showing peak gradient of 89 mm Hg, systemic
pressures were 100 mm Hgestimated pulmonary artery (PA) pressures will be 11 mm Hg; (B) Shows the peak systolic gradient across
the ventricular septal defect (VSD) of 98 mm Hg, systemic pressures were 120 mm Hg. By these observations, the predicted right
ventricular (RV) systolic pressure would be 22 mm Hg.
Figs 72.32A to C: Two-dimensional transesophageal echocardiography. (A) Four-chamber view showing atrial and atrioventricular
valve rims (arrows); (B) Basal short-axis view showing the atrial and
aortic rims (arrows); (C) Modified basal long-axis view, showing the
superior vena caval and inferior vena caval rims (arrows).
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Step-wise Approach
(On Echocardiography)
Step-wise approach (on echocardiography) for evaluation
of any shunt lesion involves:
Determining the presence of shunt lesion (Fig. 72.33).
Defining its location and size of defect (Figs 72.32A to C).
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Initial indication is the volume overload of the chambers [right atrium (RA) and right ventricle (RV)] in 4C view
Visualize the defect from subcostal view (sagittal and coronal views)
Determine the site of defect: fossa ovalis or other
Determine the direction of shunting
Assess associated structures, particularly pulmonary veins and atrioventricular (AV) valves
Assess pulmonary artery pressures: tricuspid regurgitation (TR) and pulmonary regurgitation (PR) gradients
Determine suitability for device closure
Echocardiographic Imaging in
Atrial Septal Defects
Enlarged RA, RV, and paradoxical septal motion of
interventricular septal motion are indirect evidence of
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Figs 72.35A and B: Two-dimensional echocardiography. Subcostal bicaval view with color comparison showing fossa ovalis atrial
septal defect with adequate superior vena cava (SVC) rim (upper
arrow) and deficient inferior vena cava (IVC) rim (lower arrows) and
left-to-right shunt. (LA: Left atrium; RA: Right atrium; SVC: Superior
vena cava).
Figs 72.36A and B: Two-dimensional echocardiography. Subcostal bicaval view with color comparison showing the superior vena cava
(SVC) type of sinus venosus atrial septal defect. SVC is overriding the defect with partial anomalous pulmonary venous drainage of
right upper pulmonary vein to SVC. (LA: Left atrium; RA: Right atrium; RUPV: Right upper pulmonary vein; SVC: Superior vena cava).
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Fig. 72.38: Two-dimensional echocardiography. Apical fourchamber view showing a large ostium primum atrial septal defect
(ASD) defect in lowermost part of the interatrial septum (arrow) with
absence of offsetting of the atrioventricular (AV) valves (arrow). (LA:
Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Direction of Shunt1416
With an isolated uncomplicated ASD, pressure between
the two atria is similar with a variation up to 5 mm Hg, with
cardiac cycle and phases of respiration. Dominant shunt
occurs from left to right. Left-to-right shunt occurs mainly
during mid to late systole as the v-wave of LA is larger
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Figs 72.39A and B: A case of total anomalous pulmonary venous drainage in a 2-year-old child. Two-dimensional echocardiography
with color compare in subcostal sagittal view showing right-to-left shunt across atrial septal defect (arrow). (LA: Left atrium; RA: Right
atrium).
V CSA RR
1000 mL /1
stroke volume.
mean velocity (velocity time integral).
Cross-sectional area of flow (cm2).
R-to-R interval (s/beat).
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Initial indication is the volume overload of the chambers [left atrium (LA) and left ventricle (LV)]
Visualize the defect from all possible windows
Determine the presence of additional defects (screening the septumseptal sweep in subcostal, apical four-chamber, parasternal
short- and long-axis views in color and B-mode)
Determine the direction of shunting through the defect
Assess associated defects, particularly outflow obstructions and adjacent structures
Assess pulmonary artery pressures: ventricular septal defect (VSD) gradients, tricuspid regurgitation (TR), and pulmonary regurgitation (PR) velocities
Determine the volume overload of chambers (z-scores of LV, particularly in M-mode)
Determine suitability for device closure or surgery
Four-chamber View
Four-chamber view can be obtained by keeping the
endoscope at the lower part of esophagus. This view
profiles the atrial septum with fossa ovalis defect in the
middle of septum, atrial and AV valve rims, and volume
overloaded RA and RV. By rotating the endoscope, we
can see the attachment of right and left pulmonary veins
draining into LA. In this view mitral valve morphology and
mitral regurgitation can also be assessed.
Confirmation of VSD.
Determination of the size and morphological location
of VSDs.
Ruling out associated lesions.
Assessment of chamber size and wall thickness.
Estimation of shunt size (pulmonary/systemic flow
ratio).
Estimation of RV and pulmonary arterial pressures.
Morphological Location17,18
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Figs 72.41A and B: Two-dimensional echocardiography. (A) Apical four-chamber view with anterior tilt showing the perimembranous
ventricular septal defect (VSD) (arrow) getting restricted by septal leaflet of the tricuspid valve in two dimensions; (B) Color flow mapping of the same patient showing the turbulent jet of the restricted VSD with small left ventricle (LV)-to-right atrium (RA) shunt (arrow).
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Muscular inlet defects: These defects lie within the boundaries of the smooth inlet septum and will be visualized by
scanning through the four-chamber plane. They are not
visualized in any other of the echocardiographic planes,
and their characteristic feature, which allows differentiation from the perimembranous inlet defects, is that the
upper muscular rim of the defect is separated from the AV
junction by a muscle bar. Thus, in muscular inlet defects,
the AV junction morphology is normal compared to the
abnormal morphology in perimembranous inlet defects.
Figs 72.42A and B: Two-dimensional echocardiography in subcostal coronal view (A) with anterior tilt and color compare; (B) Showing
outlet muscular ventricular septal defect (VSD; arrow). (LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).
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Figs 72.43A and B: Two-dimensional echocardiography with color compare in parasternal long-axis view showing the apical muscular
ventricular septal defect (VSD) with left-to-right shunt.
Figs 72.44A and B: Two-dimensional echocardiography with color compare in parasternal long-axis view showing the doubly committed
ventricular septal defect (VSD) with left-to-right shunt.
Fig. 72.45: Two-dimensional echocardiography in apical fourchamber view showing the large inlet ventricular septal defect
(VSD) getting partially restricted by septal leaflet of tricuspid valve
(arrow).
1595
Figs 72.46A and B: Two-dimensional echocardiography. (A) Subcostal coronal view with anterior tilt showing the perimembranous
ventricular septal defect (VSD); (B) Parasternal long-axis view with posterior tilt toward the tricuspid valve and color compare showing
the perimembranous VSD.
1596
Doppler Evaluation of
Ventricular Septal Defect
Color Doppler
Use of color Doppler has proved a valuable addition to the
diagnosis of VSDs in several ways.
The location of VSDs especially smaller ones and
multiple muscular ones can easily be determined
using Doppler color flow mapping technique.
Size of VSD.
Determination of shunt direction across the VSD.
Color flow mapping is also useful for proper alignment
of jet flow for Doppler interrogation.
Color flow mapping has a major role in rapid detection
and localization of VSDs (Fig. 72.49).2528 Small ventricular
septal defects, especially muscular, can be missed by
2D echocardiography. Color flow mapping is sensitive
in detecting small and multiple VSDs. The sensitivity of
color flow mapping is more with restrictive defects than
nonrestrictive ones, probably because of early detection of
a turbulent jet with a restrictive VSD.
Color flow mapping has been used to define the
direction of shunt across a VSD whether it is left-to-right
Figs 72.48A to C: Two-dimensional echocardiography. (A) Parasternal long-axis view showing perimembranous ventricular septal defect
(VSD) with anterior malalignment of the septum (arrow); (B) Subcostal coronal view with anterior tilt showing the anterior malalignment
of the septum (arrow); (C) Parasternal long-axis view showing perimembranous VSD with posterior malalignment of the septum (arrow).
1597
Fig. 72.49: A 25-year-old man with Eisenmenger syndrome. Twodimensional echocardiography with parasternal long-axis view
showing doubly committed ventricular septal defect with right-toleft shunt. (LV: Left ventricle; RV: Right ventricle).
1598
Fig. 72.51: Two-dimensional echocardiography in apical fourchamber view with color compare showing the well-opened mitral
valve (arrow) and turbulence across mitral valve on color flow
mapping, because of increased flow across the mitral valve in
a patient with nonrestricted perimembranous ventricular septal
defect. (LA: Left atrium; LV: Left ventricle).
Utility of Transesophageal
Echocardiography
TEE has helped in better visualization of VSDs when the
transthoracic window is poor, especially in adolescents
and adults. Straddling and overriding of the AV defect can
be better detected by TEE. TEE is extremely useful during
percutaneous closure of these defects.
Echocardiography1,2,2931
Objectives (Table 72.6)
1599
Echocardiographic Views
The following echocardiographic views profile accurately
the morphology of the duct.
Ductal view (Fig. 72.52): The transducer is placed in the
high parasternal window just beneath the left clavicle.
After obtaining a short-axis cut of the great vessels
visualizing the PA bifurcation, the transducer is rotated
anticlockwise in gradual motion. At one point, the right PA
goes away from the view and the duct with the adjacent
descending aorta opens. This view in neonates and infants
also visualizes the origin of the left subclavian artery. In
patients with associated coarctation, the posterior shelf
and coarctation can also be well visualized in this view.
Suprasternal view: There are three views to visualize the
duct from the suprasternal view.
(1) Suprasternal long-axis viewThis is the best view for
visualizing the vertical duct arising from the undersurface
of the transverse arch in patients with pulmonary atresia.
The origin of such ducti is well seen, but the insertion point
at the PA requires further anterior tilt. This is because of
the tortuous nature of such ducti.
In patients with discordant ventriculoarterial
connection (e.g. transposition of the great vessels), the
duct can be visualized well in its entire length in this view.
(2) Suprasternal short-axis viewThis is the classical
short-axis arch view and can visualize those ducti which
arise from the base of the left subclavian artery and
descend straight down to insert into the left PA. If the aortic
arch is right-sided and the patient has pulmonary stenosis
physiology, the entire length of the duct can be seen in this
view because unlike in patients with a vertical duct, it does
not follow a tortuous course.
Visualize the ductus ostium and aortic isthmus from the parasternal short-axis, high parasternal short-axis, and suprasternal views
Determine the direction of shunt by color flow mapping and Doppler
Take the peak velocity of the patent ductus arteriosus (PDA) signal, which will give the pressure difference between the aorta and
pulmonary artery, and obtain the aortic, right ventricular outflow tract (RVOT), and pulmonary artery (PA) velocities
Perform measurements of the left ventricle and left atrium as these will reflect the volume of the left-to-right shunt
Specifically look for associated defects like coarctation of aorta (suprasternal view), subaortic membrane, bicuspid aortic valve,
aortic interruption, and aortopulmonary window (communication between the ascending aorta and pulmonary artery)
1600
Duct Morphology
Usual Ductus
Vertical Duct
A vertical duct arises from the undersurface of the aortic
arch, has a tortuous course, and is commonly seen in
patients with VSD and pulmonary atresia because of
in utero flow from the aorta to the pulmonary arteries.
The best view to define a vertical duct is suprasternal
long-axis view. This view shows the duct arising from the
undersurface of arch and having a S curve. Because
1601
Subclavian Origin
Subclavian origin of ductus occurs with duct-dependent
pulmonary blood flow with right aortic arch. The
suprasternal view defines best this ductus. It has a
straighter course; hence the Doppler alignment is good.
For catheter intervention, it is easy to cannulate this type
of ductus arteriosus from the femoral arterial route.
Hemodynamic Significance
Hemodynamic significance of ductus arteriosus can be
assessed by evidence of volume overload of LA and LV,
direction of shunt, and pulmonary arterial pressure.
Chamber Dimensions
Left atrial enlargement signifies increased pulmonary
venous return because of left-to-right ductal shunting. The
ratio of the LA to aorta (Ao) is measured at the level of the
aortic valve (the LA: Ao ratio) by M-mode echocardiography
in PLAX view. The aortic root does not enlarge significantly
with even an extremely large PDA. In general, LA: Ao ratio
> 1.3:1 indicates a significant shunt. The LV will enlarge
as cardiac output increases with increased pulmonary
venous return. The best method to determine the presence
of volume overload of the LV is M-mode measurement of
left ventricular diastolic dimension and comparing it with
normal values for the patients age and weight.
1602
Figs 72.53A and B: (A) Two-dimensional echocardiography in suprasternal view showing diastolic flow reversal in the arch of aorta; (B)
Same view with cursor across the flow on M-mode showing pan-diastolic flow reversal. (D. Ao: Descending aorta; TA: Transverse arch).
Limitations of Echocardiographic
Imaging of the Duct
There are several limitations of profilation of a duct by 2D
imaging.
Limited acoustic windows: The duct is profiled in the
direction of lateral resolution of the transducer, hence
it is difficult to visualize with certainty a very small
duct in small babies. A high frequency probe with an
excellent lateral resolution is helpful.
If the duct is long and tortuous, it may be difficult to
profile the whole length of the duct.
Poor acoustic windows in adolescent and adult
patients, deformity, hyperinflated lungs, very obese
children.
Limitations in Hemodynamics
The long length of a PDA results in underestimation of
pressure gradients across it.
AORTOPULMONARY WINDOW
(FIGS 72.54A AND B)
Aortopulmonary window (APW) or aortopulmonary septal
defect accounts for 0.2 to 0.6% of patients with congenital
heart defects.34 Nearly half of all patients have associated
cardiac lesions, including aortic origin of the right PA,3539
Types
APW is classified into three types:37
Type I: Defect between semilunar valves and PA bifurcation.
Type II: Distal type defect involving origin of right PA.
Type III: Large defect involving both type I and type II.
Echocardiography: Objectives are:
Diagnosis
Type of APW
Associated heart defects
Operability
2D echocardiography usually can accurately diagnose
the aortopulmonary septal defect. Views most useful
for profiling an APW are subcostal coronal view, fivechamber view, PSAX plane at the level of great vessels,
and the suprasternal views. In all these views, the wall
separating the aorta and PA is aligned in the direction of
lateral resolution, so great care is needed to differentiate
a true defect from an artifactual dropout. A T artifact at
the edges of the defect will distinguish it from a normal
dropout and CFI will confirm the defect.
1603
Figs 72.54A and B: Two-dimensional echocardiography with color comparison in parasternal short-axis view showing a large aortopulmonary window (arrow). (Ao: Aorta; PA: Pulmonary artery).
Figs 72.55A and B: Two-dimensional echocardiography with color mapping showing the Gerbode defect (arrow) with left-to-right shunt.
(Ao: Aorta; RA: Right artium; LV: Left ventricle).
GERBODE DEFECT
(FIGS 72.55 AND 72.56)
In this entity, the shunt occurs from LV to RA through a
defect in the membranous ventricular septum and essentially leading to volume overload of the RA and the RV.
In these cases, RA dimensions and features of right
atrial and ventricular volume overload need to be
evaluated. The features are essentially same as ASD.
The point to remember is that the pressure difference
across the tricuspid valve taken in these circumstances
may be fallacious as one may mistake the high velocity LV
to RA jet for the tricuspid regurgitation velocity resulting in
an erroneous diagnosis of severe PA hypertension.
1604
Figs 72.56A and B: Two-dimensional echocardiography with color comparison in parasternal short-axis view showing Gerbode defect
(arrow) with left-to-right shunt (arrow). (Ao: Aorta; LA: Left atrium; RA: Right atrium; RV: Right ventricle).
Types Of Atrioventricular
Septal Defect
Partial Atrioventricular
Septal Defect (Fig. 72.61A)5156,59,60
Subcostal coronal and sagittal views supplemented with
apical four-chamber, PLAX and short-axis views can
define the anatomy with great accuracy. In addition, AV
valves need to be profiled in subcostal en face view by
rotating the transducer 30 to 45 clockwise from subcostal
four-chamber view. From this view, with tilting the plane
from anterior to posterior, all five leaflets, separate AV
valve orifices, attachment of anterosuperior bridging
leaflet to anterior muscular septum, and posteroinferior
septum to inlet septum can be defined. The opening of
AV valves is seen as two separate openings created by the
1605
Fig. 72.57: Two-dimensional echocardiography. Subcostal paracoronal view with anterior tilt showing the components of the common atrioventricular (AV) valve. (Ao: Aorta; IBL: Inferior bridging
leaflet; LV: Left ventricle; RV: Right ventricle; SBL: Superior bridging leaflet).
Fig. 72.59: Two-dimensional echocardiography in apical fourchamber view showing large inlet ventricular septal defect (VSD;
arrow) in a case of atrioventricular septal defect. (LA: Left atrium;
LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
1606
Figs 72.61A and B: Two-dimensional echocardiography. (A) Apical four-chamber view showing a large ostium primum atrial septal
defect (ASD; arrow), no ventricular septal defect (VSD), lack of offsetting of the atrioventricular (AV) valves (arrow); (B) Apical fourchamber view showing a large ostium primum ASD and a large inlet VSD. (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV:
Right ventricle).
1607
Table 72.8: While Evaluating a Patient with Atrioventricular Septal Defect, the Following Needs to be Addressed
1608
Figs 72.62A and B: Two-dimensional echocardiography. Apical four-chamber view showing unbalanced atrioventricular canal defect
with left ventricle (LV) dominance (A) and right ventricle (RV) dominance (B). (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV:
Right ventricle).
1609
Table 72.9: Left Ventricular Outflow Tract Obstruction in Atrioventricular Septal Defect
Tight adherence of superior bridging leaflet to septal crest causing left ventricular outflow tract to be longer and narrower. Left
ventricular outflow tract obstruction is more common in partial atrioventricular septal defect and with primum ASD
Discrete subaortic membrane
Ventricular septal hypertrophy
Abnormal chordal attachment of superior bridging leaflet
Prominent anterolateral muscle bundle
Left ventricular outflow tract can be profiled from subcostal coronal view with anterior tilt, subcostal sagittal view, and parasternal long-axis view. Color flow mapping shows turbulence beginning in subaortic area
Careful Doppler interrogation shows site of obstruction and severity of obstruction of left ventricle outflow tract
Figs 72.63A to D: (A) Subcostal en face view profiling the common atrioventricular (AV) valve completely; (B) Tracing the common
AV valve orifice during end diastole, averaged over three cardiac cycles (area B); (C) This circumference is then divided by a line drawn
over the interventricular septum from the tip of the infundibular septum to the crest of the muscular septum, thus dividing the AV valve
into left and right components (D) Take the area of the left component. For a balanced atrioventricular septal defect, AVVI (A area/B
area in figure) should be > 0.67.
1610
Hemodynamic Assessment of
Atrioventricular Septal Defect
Without Pulmonary Stenosis
Partial atrioventricular septal defect: These defects
behave like an ASD. In the absence of significant AV
valve regurgitation and normal PA pressure, the lesion
is well tolerated and patients may present late like fossa
ovalis ASDs. Significant AV valve regurgitation, however,
may cause early congestive heart failure. Accurate
assessment of PA pressure and AV valve regurgitation is
critical in decision-making for timing of surgery. Doppler
assessment of PA pressure is usually performed by
assessing tricuspid regurgitation velocity. Care should be
taken in not confusing LVright atrial shunt for tricuspid
regurgitation as the former (LVright atrial shunt) will
invariably produce high velocity signals which will not be
reflecting pulmonary arterial pressures.
Complete atrioventricular septal defect: This is associated with large VSD and pulmonary arterial hypertension. Thus, congestive heart failure develops in
the first few months of life. Also, rapid progression
(6 months of life) of pulmonary vascular disease occurs
in this condition. Thus, there is an urgent need to correct
these lesions early in life. If correction is performed at
the appropriate age, then echocardiography alone is
enough for assessment of this lesion as the morphology
is well delineated by this technique and there is no need
for invasive determination of PA pressure and vascular
resistance. Late presentation, however, may need more
detailed evaluation with cardiac catheterization.
Mechanism of Atrioventricular
Valve Regurgitation
In the majority of cases, AV valve regurgitation occurs
through the cleft in the left AV valve. This is well-appreciated in the PSAX views and subcostal paracoronal
(en face) view. Regurgitation can also occur through the
commissures of the left AV valve or through the right
AV valve.
1611
Infective
Myocarditis
Kawasaki disease
Infective endocarditis
Storage disease/infiltration
Hurler disease
Amyloidosis
1612
Fig. 72.66: Two-dimensional (2D) echocardiography from an infant with cor triatriatum. Zoomed up apical four-chamber view on
2D echocardiography, showing a shelf in left atrium stretching
from atrial septum on the right side to lateral wall of left atrium on
the left with a narrow communication (arrow). (LA: Left atrium; LV:
Left ventricle; RA: Right atrium; RV: Right ventricle).
Fig. 72.67: Two-dimensional echocardiography. Apical four-chamber view with color flow mapping showing turbulence (arrow) in
a case of cor triatriatum. (LA: Left atrium; LV: Left ventricle; RA:
Right atrium; RV: Right ventricle).
1613
1614
Figs 72.70A and B: Two-dimensional echocardiography in apical four-chamber view (A) showing double orifice mitral valve with separate subvalvular apparatus; (B) Parasternal short-axis view at the level of the mitral valve showing the two separate orifices of equal
size. (LA: Left atrium; LV: Left ventricle).
1615
1616
Ebsteins anomaly
Tricuspid valve dysplasia
Tricuspid valve prolapse
Double orifice tricuspid valve
Parachute deformity
Congenitally unguarded tricuspid orifice
Tricuspid atresia
CONGENITAL ABNORMALITIES OF
TRICUSPID VALVE (TABLE 72.11)
Apart from Ebsteins anomaly, other congenital anomalies
of the tricuspid valve apparatus (valve annulus, valve
leaflets, chordae tendineae, and papillary muscles) are not
very common. Dysplastic valve with varied abnormalities
can occasionally be seen.
Figs 72.71A and B: Two-dimensional echocardiography in a patient with Ebsteins anomaly of tricuspid valve. (A) Apical four-chamber
view with slight leftward tilt showing the apical displacement of the septal leaflet of tricuspid valve (arrow), (+) shows the normal site
of attachment of TV, enlarged right atrium, atrialized right ventricle, and reduced size of the functional right ventricle; (B) Apical fourchamber view with posterior tilt (at the plane of coronary sinus) showing displaced posterior leaflet of tricuspid valve. (ARV: Atrialized
right ventricle; CS: Coronary sinus; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
1617
Congenitally Unguarded
Tricuspid Orifice1,2,112
Here, the orifice between the RA and the RV is normal,
but there is no tricuspid valve apparatus. There is either
complete absence of valve or only remnants of valvular
tissue are present. The close differential includes dysplastic
tricuspid valve (Figs 72.72A and B). Usually, the RA is
dilated and the RV is hypoplastic.
This anomaly is best visualized in apical and subcostal
four-chamber views as well as in long-axis parasternal
inflow view. Severe low-pressure tricuspid regurgitation
is invariably present. Associations include pulmonary
Figs 72.72A and B: Two-dimensional transthoracic echocardiography. Apical four-chamber view with color compare in a case of
severely dysplastic tricuspid valve showing markedly enlarged right atrium and severe tricuspid regurgitation. (LA: Left atrium; LV: Left
ventricle; RA: Right atrium; RV: Right ventricle).
1618
Figs 72.73A to C: Two-dimensional transthoracic echocardiography in a case of Uhls anomaly. (A) Apical four-chamber view
showing dilated thin walled RV (arrow) and dilated tricuspid orifice
with tricuspid valve (arrow). (B) Parasternal short-axis view with
Uhls anomaly showing dilated right atrium with thinned out tricuspid valve (arrow). (C) Subcostal sagittal view of the same patient
showing thin tricuspid valve and the lack of apical trabeculations of
right ventricle (arrow).
atresia with intact ventricular septum, ASD, PDA, doublechambered RV, cor triatriatum, VSD, Uhls anomaly113
(Figs 72.73A to C), and LSVC draining into RA.
Uhl's Anomaly113
Though not a disease of the tricuspid valve it is discussed
here because it mimics the presentation of Ebstein
anomaly of TV or dysplastic TV closely. Uhls anomaly
1619
The PSAX view at the base of the heart and long-axis view
are best views to determine the morphology and number
of cusps.
Normally, the tricuspid aortic valve has three cusps
of nearly equal size. Three commissures form a Y-shaped
pattern in diastole. In systole, the leaflets open along these
commissures to create a wide-open triangular orifice. The
congenital anomalies of aortic valve comprise a spectrum
of deformities, which include a decrease or increase in
the number of valve cusps, their form, and size. Normal
aortic valve leaflets are thin with unrestricted mobility.
In stenotic valves, leaflets are thickened and domed in
systole. Doming of valve leaflets during systole occurs due
to limited cusp separation leading to restricted mobility
of valve cusps. Subcostal coronal with anterior tilt, apical
four-chamber with anterior tilt, and PLAX views are
Figs 72.74A and B: Two-dimensional transthoracic echocardiography. (A) Parasternal short-axis view showing bicuspid aortic valve in
diastole with single closure line of fusion; (B) The same patient in systole showing the fused right and noncoronary cusps of the aortic
valve (arrows). (LA: Left atrium; RA: Right atrium; RV: Right ventricle).
1620
Figs 72.75A and B: (A) M-mode cut across the aortic valve and left atrium showing eccentric closure of the thickened aortic valve; (B)
Showing the same across the normal valve for comparison.
1621
Sinotubular Junction
Sinotubular junction is the point of the union of the aortic
sinuses and the tubular portion of the ascending aorta.
The apex of the aortic valve commissures correspond to
the sinotubular junction.
PLAX view is the best view to measure the aortic
valve annulus, the thickness and mobility of the leaflets,
the plane of the valvular orifice, the sinuses of Valsalva,
the sinotubular junction, and the proximal portion of the
ascending aorta.
1622
122128
Pressure Gradients
CSAav =
CSALvot VTILvot
VTIav
1623
Hemodynamics
The LV becomes hypertrophied with increasing left ventricular outflow obstruction. Severe unrelieved obstruction may lead to an oxygen demand/supply mismatch
leading to subendocardial ischemia and fibrosis.
Ventricular systolic function is assessed by the
conventional methods of calculating shortening fraction
and ejection fraction. This is an important parameter in
the echocardiographic assessment of an aortic stenosis
patient as the assessment of severity by pressure gradients
depends upon ventricular function. A decrease in function
decreases the transvalvular flow and the gradients no
longer reflect the severity of obstruction.
Diastolic ventricular function is assessed by the filling
abnormalities of the LV. From the mitral valvular Doppler
recording peak flow velocities, filling rates and proportion
of flow in various phases of diastole may be assessed.
Comparative studies of these subjects with normal controls
have revealed higher E-velocity, a much higher A-velocity,
therefore an inverse E/A ratio. The percentage of total
Doppler area in the first third of diastole was significantly
lower and the percentage of the total Doppler area under
the A-wave was higher. M-mode tracing in PLAX view at
the mitral valve may show a b-bump or a c-interruption if
the LVEDP is elevated.
1624
Associated Anomalies129-135
A PDA is seen in 20 to 65% cases of valvular aortic stenosis.
CoA is found in 11 to 53% cases and stenosis of the mitral
valve in 25% cases. Other anomalies like VSD and mitral
valve abnormalities are also common and should be
looked for.
Fig. 72.80: Two-dimensional echocardiography. Parasternal longaxis view in a child with discrete subaortic membrane showing
anterior insertion of the membrane to the ventricular septum below
the right aortic cusp (arrow). (Ao: Aorta; LA: Left atrium; LV: Left
ventricle; RV: Right ventricle).
1625
Fig. 72.81: Two-dimensional echocardiography. Parasternal longaxis view in a 2-year-old child with discrete circumferential subaortic membrane (arrows) close to aortic valve. (Ao: Aorta; LA: Left
atrium, LV: Left ventricle; RV: Right ventricle).
Fig. 72.82: Two-dimensional echocardiography. Parasternal longaxis view in a child with hypertrophic obstructive cardiomyopathy
showing the severely hypertrophied interventricular septum and
systolic anterior motion of the anterior leaflet of the mitral valve
(arrow). (LA: Left atrium; LV: Left ventricle; RV: Right ventricle
Ao: Aorta).
1626
Fig. 72.83: Two-dimensional echocardiography. Apical fourchamber view with anterior tilt and color flow mapping in a case
of supravalvular aortic stenosis. The turbulence (arrow) begins
above the level of the aortic valve (arrow). (AA: Ascending aorta;
LV: Left ventricle).
Morphology
The obstruction is best imaged in parasternal long-axis,
apical five-chamber and subcostal views of the LVOT, or
suprasternal long- and short-axis views and high right
parasternal views. In the parasternal and apical fivechamber views in the normal heart, the aortic diameter
increases at the level of the sinuses of Valsalva and then
decreases at the superior border of the aortic sinuses. The
diameter of the ascending aorta above the aortic sinuses is,
however, the same as the diameter of the ventriculoaortic
junction or aortic root in the normal heart.
The detection of obstruction in hourglass deformity
depends upon visualization of an obvious decrease
in the caliber of the vessel relative to the surrounding
normal areas, namely at sinotubular junction, then
some dilation, and narrowing again. The following
measurements are taken in the PLAX view: (a) aortic
annulus, (b) maximal diameter at sinuses of Valsalva, (c)
sinotubular junction, (d) narrowest part, and (e) aorta
distal to the obstruction.
Measurements are taken from the inner aspect of
the aortic root echo to the inner aspect of the posterior
root echo. Careful attention should be paid to aligning
the probe so that long axis of the vessel and adequate
visualization of the lumen both proximal and distal to the
area of obstruction is obtained. Oblique angulation of the
cross-sectional scan plane to vascular lumen may give
the appearance of the aorta being cut-off as beam passes
obliquely through the lateral wall of the vessel.
Normally, the diameter of the sinotubular junction is
either equal or slightly (12.5%) more than the diameter
of the aortic annulus. In supravalvular aortic stenosis, a
percentage decrease of > 25% from the annulus is noted.
There is a rough correlation in the percentage decrease
1627
1628
Coronaries
Coronary artery abnormalities are invariably associated
and may be:
Dilated and tortuous coronary arteries due to
exposure to high systolic pressures proximal to the site
of obstruction (rarely coronary artery aneurysms have
been reported)
Coronary orifice can be narrowed by the overhanging,
fibrous bridge.
Rarely, the orifice of coronary artery can be completely
obstructed as the valve cusps become adherent to the
aortic wall.
With the patient in the left lateral position, the
transducer is placed in the left parasternal position
to obtain a short-axis view of the great vessels. Slight
adjustment of the imaging plane, so that it traverses the
heart below the pulmonary trunk, allows for visualization
of the origin and proximal portion of coronary arteries.
The ostia and the coronaries should be carefully assessed
for dilatation, tortuosity, or narrowing. The apical fourchamber view demonstrates RCA coursing along the right
AV groove and aiming the transducer superiorly toward
the left ventricular outflow, the branching of left anterior
descending (LAD), and left circumflex may be seen
besides their profiling in the PSAX view. In severe cases,
myocardial ischemia and infarction have been reported
as such regional wall motion abnormalities should be
assessed at echocardiography.
Serial Echocardiograms
The condition is usually progressive in nature and cases
with mild narrowing need careful long-term follow-up.
AORTIC REGURGITATION
Congenital aortic insufficiency is a rare entity as an
isolated lesion. It frequently occurs in association with
Truncus arteriosus
1629
Figs 72.86A and B: Two-dimensional echocardiography. Parasternal long-axis view with slight anterior tilt and color comparison
in a 7-day-old neonate with aortico-left ventricular tunnel (arrow). Color flow mapping shows aortic regurgitation. (Ao: Aorta; RV: Right
ventricle; LV: Left ventricle; T: Tunnel).
1630
Figs 72.87A and B: Two-dimensional echocardiography a case of annulo-aortic ectasia in a 2-year-old girl. (A) Parasternal long-axis
view showing the dilated (ectatic) aortic root; (B) Subcostal coronal view with anterior tilt showing the left ventricular outflow tract with
ectatic aortic root in the same patient. (Ao: Aorta; RV: Right ventricle; LV: Left ventricle; LA: Left atrium).
Cusp prolapse
Vegetations
Severity of Regurgitation
With the use of 2D echocardiography, left ventricular
dilatation and function can be assessed, and on color flow
1631
Figs 72.88A and B: Ruptured sinus of Valsalva in a 30-year-old male patient. (A) Two-dimensional echocardiography with color compare in parasternal short-axis view showing aneurysm of noncoronary sinus rupturing into the right atrium (arrow). (Ao: Aorta; LA: Left
atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Aortic Regurgitation
Aortic regurgitation is a frequent occurrence in patients
with an aneurysm of sinus of Valsalva with or without
rupture. Aortic regurgitation occurs due to loss of support
of the aortic sinus and its annulus and its distortion. In
Associated Anomalies
Deformation and rupture of the sinus of Valsalva is
commonly associated with VSD, so it has to be looked
for carefully (Fig. 72.91). The right sinus may prolapse
through a doubly committed VSD towards RVOT, causing
RVOT obstruction and subsequent rupture. Both the right
coronary and noncoronary sinuses may prolapse through
a perimembranous VSD into RV inflow, which may lead to
distortion of tricuspid valve and tricuspid regurgitation.
Other associated anomalies are pulmonary stenosis, TOF,
ASD, bicuspid aortic valve, CoA, and LSVC, which needs to
be scanned carefully.
Aneurysm of sinus of Valsalva can be best defined
from parasternal long-axis, subcostal coronal and apical
four-chamber views with tilting the transducer toward RV
inflow and outflow. PSAX view also defines the anatomy
with the sinus protruding into RV inflow, outflow, RA,
LA, or into the PA. Combination of 2D echocardiography
with color flow mapping and pulsed/continuous wave
Doppler interrogation is helpful in detailed profilation
of the lesion. With rupture of aneurysm into right-sided
chambers, there will be dilatation of right-sided chambers
with large left-to-right shunt and rise of pressures. With
1632
AORTOCAMERAL
COMMUNICATIONS168174
Aortocameral communications are abnormal communications between the root of aorta and one of the cardiac
chambers, the commonest being aortico-left ventricular
tunnel, followed by aortico-right atrial tunnel, aortico-RV
tunnel, and least in frequency is aortico-LA tunnel.
1633
Commonest VSD seen in 74% of cases of TOF is perimembranous outlet VSD, since the tricuspid valve forms
one of the margins of the defect. Echocardiographically,
the subcostal sagittal and coronal views show tricuspid
aorticmitral continuity, which is a differentiating feature
from DORV. The aortic valve forms the roof of the defect;
the posterior-inferior margin is the area of tricuspid
aorticmitral continuity and the anterior and anteriorinferior margins are muscular. The PSAX view at the level of
the aortic valve shows that the VSD extends from the area
of the tricuspid valve anteriorly to the area of the muscular
septum (Fig. 72.91). The outlet septum itself separates the
aortic valve from the pulmonary valve.
Muscular outlet VSD is seen in 20% of cases of TOF. A
muscle bar is present in the posterior-inferior margin of
the defect and this separates the tricuspid valve from the
aortic valve. Thus, except for the superior margin, which is
formed by the aortic valve, the VSD in rest of the margins
is entirely muscular. Echocardiographically, this feature is
well seen in the subcostal coronal view as a bar of muscle separating the aortic valve from the tricuspid valve. In
the PSAX view also the aortic valve is separated from the
tricuspid valve by a muscle bar. The importance of recognizing this entity is that this bar of tissue separates the conduction tissue from the margins of the defect making the
chances of heart block unlikely when the VSD is closed.
AORTIC OVERRIDE
Figs 72.89A and B: Two-dimensional echocardiography in parasternal long-axis view with color comparison in a 2-year-old child with
tetralogy of Fallot showing a large perimembranous ventricular septal defect (VSD), aortic override ( ), and aortic mitral continuity
(arrow). (Ao: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
1634
Fig. 72.90: A 2-year-old child with tetralogy of Fallot. Two-dimensional echocardiography with subcostal paracoronal view showing
the large perimembranous ventricular septal defect (VSD; arrow)
with anterior malalignment of the septum, causing subvalvular pulmonary stenosis. (Ao: Aorta; RVOT: Right ventricular outflow tract;
RV: Right ventricle).
Pulmonary Stenosis181185
RVOT narrowing in a case of TOF is a consequence of
the anterior and cephalad deviation of the infundibular
septum. The hallmark in TOF is infundibular stenosis
with variable degree of valvular stenosis (Figs 72.97
and 72.98). Thus, patients with large VSD and isolated
valvular stenosis should not be classified under TOF.
Anterocephalad deviation of the outlet septum is
seen best echocardiographically in the subcostal
sagittal, coronal views and PSAX views at the level of
the great arteries. In patients of TOF and pulmonary
atresia, the deviation is so extreme so as to produce
1635
Fig. 72.92: Two-dimensional echocardiography. Parasternal shortaxis view in a 3-year-old child with tetralogy of Fallot and doubly
committed ventricular septal defect showing the point of continuity
between aortic and pulmonary valves (arrow). (Ao: Aorta; PA:
Pulmonary artery; RV: Right ventricle).
Fig. 72.94: Two-dimensional echocardiography. Modified parasternal short-axis view in a case of tetralogy of Fallot showing the
additional muscular ventricular septal defect (VSD) tract (arrow)
with left right shunt. (LV: Left ventricle; RV: Right ventricle).
1636
Figs 72.98A and B: Two-dimensional echocardiography in subcostal paracoronal view with color compare showing severe
infundibular pulmonary stenosis (arrow) with turbulent flow in
right ventricular outflow tract (RVOT), in a patient with tetralogy of
Fallot. (PA: Pulmonary artery; RV: Right ventricle).
1637
Fig. 72.101: Two-dimensional echocardiography. Modified parasternal short-axis view with color compare in a patient with
tetralogy of Fallot showing absence of pulmonary valve (arrow)
with hypoplastic pulmonary artery annulus and free pulmonary
regurgitation (on color mapping) across the pulmonary annulus.
(PA: Pulmonary artery; RV: Right ventricle).
Fig. 72.102: Two-dimensional echocardiography in high parasternal short-axis view showing a narrow origin (arrow) of left pulmonary artery in a case of TOF. (Ao: Aorta; LPA: Left pulmonary artery; MPA: Main pulmonary artery).
1638
Figs 72.103A and B: Two-dimensional echocardiography with color compare in high parasternal short-axis view showing confluent
branch pulmonary arteries with diffuse narrowing in left pulmonary artery (LPA, arrow). (MPA: Main pulmonary artery; RPA: Right
pulmonary artery).
and left PA, (b) the site of insertion of the patent ductus
arteriosus into the left PA, and (c) supravalvular narrowing
of the main PA. Diffuse hypoplasia can involve the left or
right pulmonary arteries or both. Multiple, peripheral PA
narrowing within the lung parenchyma is very rare and
cannot be detected by echocardiographic scans.
Aortic Arch
The suprasternal view is used to differentiate left and right
aortic arches. Right aortic arch is seen in 25% of patients
with TOF (Fig. 72.106). Two methods are used for their
differentiation: (a) In the suprasternal short-axis view,
the transducer is tilted posteriorly. In right aortic arch,
the descending aorta mostly falls to the right and in left
aortic arch the descending aorta mostly falls to the left.
(b) The branching pattern of the head and neck vessels is
most important. In left aortic arch, the normal branching
pattern of the first branch of the aortic arch (innominate
artery) can be seen dividing into two branches to the
right. The opposite is true in patients with right aortic arch
with mirror image branching. The fallacy of the second
method is when patients of right arch have an aberrant
left subclavian (3%) artery and vice versa (<1%). (c) The
1639
Figs 72.107A and B: Two-dimensional echocardiography. (A) Suprasternal long-axis view showing the origin of a collateral from the
ductal area (arrow); (B) Subcostal sagittal view showing collaterals arising from the descending aorta (arrow). (Des Ao: Descending
aorta; TA: Transverse arch).
1640
Echocardiographic Measurements in
Tetralogy of Fallot1,2,187
Fig. 72.108: Continuous wave Doppler signal showing right ventricular outflow gradient in a case of tetralogy of Fallot (TOF). Note
the sickle-shaped signal of the infundibulum stenosis (arrow).
1641
Postoperative Evaluation of
Tetralogy of Fallot
1642
Figs 72.113A and B: Two-dimensional echocardiography in parasternal long-axis view with color flow mapping in cases of tetralogy of
Fallot after total correction showing significant residual ventricular septal defect (VSD) from the upper end of the VSD patch (VSD patch
marked by the arrow). (Ao: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle).
1643
Fig. 72.114: Two-dimensional echocardiography. Apical fourchamber view in a patient with tetralogy of Fallot with transannular
patch showing dilatation of the right atrial (RA) and right ventricular (RV) cavity. (LA: Left atrium; LV: Left ventricle).
1644
Figs 72.118A and B: Right ventricle (RV) fractional area and fractional area change. Measuring the right ventricular area in diastole
(RVD) (A) and subtracting from it the right ventricular area in systole (RVS); (B) The difference divided by the right ventricle area in
diastole gives the fractional area change, a measure of the right ventricular systolic function.
Figs 72.119A and B: Right ventricular diastolic dysfunction. Showing the profiling of inferior vena cava (IVC) from subcostal window:
(A) IVC is dilated; (B) M-mode cursor across the IVC shows no respiratory phasic variation.
1645
Definition
The diagnosis of DORV (Fig. 72.120) requires the following
criteria to be fulfilled.188193
DORV infers that both great arteries arise from the RV
as the name implies. There is a spectrum of the degree of
override of one great vessel and herein lies the controversy
as to when to classify an origin as double outlet versus
concordant ventriculoarterial connection. Some authors
follow the 50% rule, which means that if a vessel is 50% or
more committed to a chamber, it is considered originating
from that chamber. However, it is difficult to determine
when exactly an override is 50% or more. To avoid this
difficulty, some authors diagnose DORV if the override is
90% or more.
Some consider absence of fibrous continuity between
the posterior semilunar valve and the mitral valve
(Fig. 72.120A). According to some, this is not mandatory.
One can have fibrous continuity of the mitral and one
semilunar valve with criteria of origin of great vessel being
fulfilled. We believe that both features are important for
the diagnosis of DORV, that is, more than 50% override and
mitral aortic discontinuity.
The VSD is located just below the aortic conus and thus
can be routed to aorta. In this case, saturations are better
because saturated blood from LV is directed to the aorta. In
the event that the great artery relationship is normal with
no pulmonary stenosis, the condition may be corrected
like a perimembranous VSD. In presence of pulmonary
stenosis, it can be repaired like a TOF. In the majority of
cases in this group great vessels are normally related. In
rare cases, the aorta may be L-malposed.
Doubly committed
Here, the conal septum between the aortic and pulmonary
outflow tract is deficient. As a result, the VSD is located
below both outflow tracts and can be routed to either great
vessel.
Remote
Remote VSDs are typically located in the inlet septum
(Fig. 72.122). The tricuspid valve tensor apparatus comes
in the path between the VSD and either of the outflow
tracts. Overriding/straddling of tricuspid valve can occur
1646
Fig. 72.122: Two-dimensional echocardiography. Apical fourchamber view in a case of double outlet right ventricle (DORV)
showing large inlet ventricular septal defect (VSD). (LA: Left
atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Figs 72.123A and B: Two-dimensional echocardiography in a subxiphoid sagittal view in two cases, showing a ventricular septal defect
located in the inlet septum (arrow) and not related to either great artery origin. (Ao: Aorta; LV: Left ventricle; PA: Pulmonary artery;
RV": Right ventricle).
Establishing a Diagnosis of
Double Outlet Right Ventricle by
Echocardiography194
The following needs to be specifically evaluated:
VSD
Subpulmonary outflow tract
Subaortic outflow tract and arch
Coronary arteries.
1647
Subpulmonary Ventricular
Septal Defect (TaussigBing Anomaly)
In this condition, the aorta is anterior (malposed) in
majority of cases. In the subcostal sagittal view, the great
vessel overriding the septum is the PA. The aorta will not be
seen or will come under view in a more rightward sweep.
In the coronal section, both great vessels will be seen
having a parallel origin with the aorta to the right and PA
to the left. Pulmonary valve and mitral valve discontinuity
will also be obvious.
1648
Table 72.14: The Complete Checklist for Preoperative Assessment of Double Outlet Right Ventricle
Fig. 72.125: Two-dimensional echocardiography. Subcostal sagittal view in a case of situs inversus with mesocardia showing a
large ventricular septal defect with hypoplasia of the right-sided
left ventricle. (Ao: Aorta; LV: Left ventricle; PA: Pulmonary artery;
RV: Right ventricle).
1649
Fig. 72.127: Two-dimensional echocardiogram. Subcostal short-axis view with anterior tilt and color compare in a case of double outlet
right ventricle (DORV) with a large subaortic ventricular septal defect (VSD) and pulmonary stenosis. Presence of tricuspid valve (arrow) in the pathway from left ventricle (LV) to aorta resulting in restriction of the VSD. (Ao: Aorta; LV: Left ventricle; RV: Right ventricle).
1650
TRUNCUS ARTERIOSUS
(FIGS 72.128, 72.131 TO 72.134)
A common arterial trunk arising from the base of the
heart, and giving origin to aorta, pulmonary arteries, and
coronaries is referred to as truncus arteriosus. A single
semilunar valve is found in truncus arteriosus, and this
valve differentiates truncus arteriosus from aortic and
pulmonary valve atresia, conditions in which a single
arterial vessel also receives the entire output of both
ventricles but in which a second atretic semilunar valve
is present. Truncus arteriosus is usually associated with a
large VSD resulting from absent or deficient outlet septum.
In 25% of cases, the defect may extend to the membranous/
perimembranous area. Rarely, the VSD may be restrictive
or even absent. According to the initial classification given
by Collet and Edwards199 in 1948, truncus may be classified
as following:
Type I: Origin of a main pulmonary trunk from the
lateral aspect of the common trunk.
Type II: Left and right pulmonary arteries originate
separately from the common trunk. The origin is
close to each other, or from a common orifice,
usually located on the posterior aspect of the
common trunk.
Type III: Origin of only one PA from the common trunk,
the other PA can originate from the ductus
arteriosus or directly from ascending aorta
(MAPCA artery).
Type IV: It is defined not by the pattern of origin of
pulmonary arterial branch but rather by the
coexistence of an interrupted aortic arch or
aortic hypoplasia or preductal aortic coarctation.
Figs 72.129A and B: Two-dimensional echocardiography. Suprasternal short-axis view showing a patent left BT shunt (arrow) to left
pulmonary artery. (LPA: Left pulmonary artery); (B) Color Doppler flow mapping in the same patient showing flow through the left BT
shunt indicating its patency.
1651
Figs 72.131A to C: Two-dimensional echocardiography. (A) Subcostal coronal view with anterior tilt in a child with truncus arteriosus Type I, giving rise to aorta and pulmonary artery (arrow)
showing the presence of single outflow truncus (Tr) overriding the
ventricular septal defect (marked by star); (B) Parasternal long axis
view showing the presence of single outflow truncus (Tr) with main
pulmonary artery stump arising from the truncus ( arrow); (C) Parasternal short axis view with color flow mapping in the same patient
showing the common trunk (MPA) giving rise to both the pulmonary
arteries). (RV: Right ventricle; LV: Left ventricle; Tr: Single outflow
truncus; Ao : Aorta).
1652
Figs 72.134A and B: Two-dimensional echocardiography in high parasternal short-axis view with color compare in a case of persistent
truncus arteriosus type I with confluent branch pulmonary arteries. (LPA: Left pulmonary artery; RPA: Right pulmonary artery; T: Truncal
valve).
Echocardiography
(Figs 72.131 to 72.134)
Intracardiac anatomy usually reveals situs solitus and AV
concordance. Two balanced ventricles are usually present
and separated by a large VSD. Truncus arteriosus has
been described with tricuspid atresia, hypoplastic double
inlet ventricle, and a very rare form with discordant AV
connection.
Two-Dimensional Echocardiography201
Subcostal coronal view with anterior tilt shows a common
trunk arising from the heart, and overriding the VSD.
Subcostal paracoronal view can profile the pulmonary
artery arising from the trunk and bifurcating into right
pulmonary artery and left pulmonary artery from lateral
aspect of the trunk. Apical four-chamber view with
anterior tilt will profile the common trunk arising from the
heart and overriding the VSD. PLAX view is the best view
to profile truncal anatomy. This view shows the common
trunk arising from the heart committed to both ventricles
and overriding the large VSD. Slight posterior tilt from a
standard PLAX view shows the origin of main PA from the
posterolateral aspect of the common trunk. PSAX view at
the base of heart shows the truncal valve in cross section,
large outlet VSD, and pulmonary arteries as they arise
1653
Doppler Imaging
Color flow Doppler mapping shows regurgitation or
stenosis of truncal valve and any stenosis at the origin
of pulmonary arteries. With continuous wave Doppler,
gradient across the truncal valve and pulmonary arteries
can be recorded.
Anatomy
Figs 72.135A and B: Two-dimensional echocardiography with color compare in parasternal long-axis view with anterior tilt in a neonate
with complete transposition of great vessels (TGA) showing ventriculoarterial discordance and the presence of parallel great vessels.
There is acute posterior angulation of the pulmonary artery as it arises from the left ventricle. (LV: Left ventricle; RV: Right ventricle; Ao:
Aorta; PA: Pulmonary artery).
1654
Associated Lesions202-205
Nearly half of the patients with d-transposition of great
vessels have no other associated anomaly except a
persistent foramen ovale or ASD and PDA (Fig. 72.136).
VSD is the commonest associated anomaly (4045%);
combination of VSD and LVOT obstruction (pulmonary
stenosis) are observed in 10%, and an isolated LVOT
obstruction in approximately 5% of cases. Less commonly
encountered anomalies are AV valve abnormalities, aortic
obstructions, arch anomalies, and anomalies of systemic
and pulmonary venous connections.
Echocardiographic Evaluation
Two-Dimensional Echocardiography
The usual segmental analysis is essential as for any other
congenital cardiac anomaly. After defining the situs,
subcostal coronal and apical four-chamber views provide
the AV connection. Anterior tilt from subcostal coronal
and apical four-chamber views profiles first the posteriorly
placed great vessel that bifurcates into two, that is, PA arising
from LV with mitralpulmonary continuity (Fig. 72.135).
Further angulation profiles the anteriorly placed great
vessel with muscular infundibulum (subaortic conus),
1655
Figs 72.137A and B: Two-dimensional echocardiography in parasternal long-axis view (Figure A) and parasternal short-axis view
(Figure B) at the level of the great vessels from an infant with transposition of great vessel (TGA) showing right and anterior position
of the aorta as compared to the pulmonary artery. Arrow shows the origin of the left main coronary artery. (Aorta: Ascending aorta; PA:
Pulmonary artery). (RV: Right ventricle; LV: Left ventricle).
Figs 72.138A and B: Two-dimensional echocardiogram in subcostal coronal view with anterior tilt. (A) Shows the origin of the aorta from
the right ventricle; (B) Shows the origin of the pulmonary artery (and pulmonary artery branching) from the left ventricle. (Ao: Aorta; LV:
Left ventricle; PA: Pulmonary artery; RV: Right ventricle).
Chamber Size206210
After birth in simple (or complete) transposition of great
vessels with intact ventricular septum, with the fall in PVR
during first few weeks of life, left ventricular mass does not
1656
Figs 72.139A and B: Two-dimensional echocardiography in apical four-chamber view with anterior tilt showing the origin of pulmonary artery
(PA) from the left ventricle (LV). Further anterior tilt shows the origin of aorta (Ao) from right ventricle the (RV). Ventricular arterial discordance.
Figs 72.140A and B: Two-dimensional echocardiography in parasternal long-axis view in a 1-year-old patient with transposition of great
vessel (TGA) with intact ventricular septum. Shows the thinned out posterior wall of the left ventricle (2.7 mm). M-mode echocardiography of the same showing the regressed left ventricle (LV).
LV volume index
LV mass/volume ratio.
With intact interventricular septum, RA and RV
are always dilated as seen in apical four-chamber and
subcostal coronal views. In subcostal sagittal and PSAX
views, RV dominates with a circular configuration while
the LV takes on a crescentic shape.
In case of elevated left ventricular pressure as with
a nonrestrictive VSD, large PDA, large aortopulmonary
collaterals, significant left ventricular outflow obstruction,
or pulmonary arterial hypertension, the circular shape of
LV will be maintained with normal posterior wall thickness
and LV mass.
1657
Table 72.15: Left Ventricular Mass and Volume Indexed to Body Surface Area in m2.204
Newborns
Infants
M-mode (g)
End-diastole
Mass/Volume Index
(g/mL)
31.3 6.1
47.7 12.7
59.8 21
1.57 0.4
38.7 5
48.8 8
56.6 15
1.27 0.2
54.6 7.6
58.6 10.3
61.9 11.5
1.08 0.2
59.8 6.9
61.9 7.1
69 24
1.05 0.1
64 6
66.9 7.6
88.2 20
1.04 0.1
66.4 7.8
74.1 9.8
91.2 40
1.1 0.1
Figs 72.141A and B: Two-dimensional echocardiography showing balloon atrial septostomy with echocardiographic imaging in a case
of transposition of great vessels (TGA). (A) Subcostal sagittal view showing the catheter in inferior vena cava (IVC). (B) Subcostal coronal view showing the inflated balloon in left atrium. (B: Balloon; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
Associated Defects
Septal Defects
Interatrial Communication: Most commonly it is a patent
foramen ovale. Fossa ovalis ASD occurs only in 5% of cases
with complete transposition of the great vessels. Interatrial
communication as a site of bidirectional shunting results
in good intracardiac mixing if ASD is of adequate size.
Subcostal sagittal and coronal views are used to profile the
atrial communication and color flow mapping is used to
assess the direction of shunt, which is usually bidirectional
(left to right during systole, i.e. with an atrial v-wave,
and right to left during diastole, i.e. with atrial a-wave).
M-mode with color flow mapping across the ASD is the
best method to profile the direction of shunting. It is very
important to assess adequacy of interatrial communication
with intact ventricular septum or small VSD for better
1658
1659
1660
Coronary Arteries
The arterial switch operation for repair of complete
transposition requires the mobilization and reimplantation
of the coronary arteries. Whenever the position of the
aortic root is abnormal, the origin of the coronary arteries
deviates from those found in the normal heart. Thus,
identification of coronary artery anatomy becomes an
integral part of the preoperative echocardiographic
examination of a child with complete transposition
of great vessels. Coronary anomalies can be correctly
diagnosed preoperatively with echocardiography in 95%
of the patients. Sinuses are described from PSAX view and
labeled as left-facing sinus (left side-sinus I) and rightfacing sinus (right side-sinus II). In most cases, the orifice
of the coronary artery is situated approximately in the
middle of the sinus of Valsalva just below the sinotubular
junction; minor deviations from this central position are
found frequently.
In general, the origin and proximal course of the
coronary arteries can be profiled from modified PSAX view
at the level of great vessels and apical four-chamber and
subcostal coronal views. From the PSAX view at the level
of great vessels, relationship of great vessels and sinuses (I
or left-facing, II or right-facing, and noncoronary) should
be defined.
1661
1662
Figs 72.147A and B: Two-dimensional echocardiography in parasternal short-axis views in two cases of complete transposition of great vessels
(TGA) showing the origin of the coronary arteries. (A) In case A, the coronaries arise from a single stump. In case B, all the three coronary arteries
arise separately. (Ao: Aorta; LAD: Left anterior descending artery; RCA: Right coronary artery; LCx: Left circumflex artery).
1663
Figs 72.149A to C: (A) Two-dimensional echocardiography in parasternal short-axis view in an infant with TGA showing the origin
of dual right coronary artery (RCA). (A) RCA from sinus II; (B) Left
main and RCA from sinus I; (C) Apical four-chamber view with tilt
showing the presence of the left circumflex artery in the left atrioventricular (AV) groove. (Ao: Aorta; LA: Left atrium; LAD: Left anterior descending artery; LCT: Left common trunk; LCx: Left circumflex artery; LV: Left ventricle; PA: Pulmonary artery; RA: Right
atrium; RCA: Right coronary artery; RV: Right ventricle).
1664
ATRIOVENTRICULAR AND
VENTRICOARTERIAL DISCORDANCE
Congenitally corrected transposition of great vessels
(CTGA) or L-transposition of great vessels is characterized
by discordant AV connections combined with discordant
ventriculoarterial connection. Thus, with situs solitus,
morphological RA on right side is connected to
morphological LV on right side (L-looped ventricles),
which is in turn connected to PA.220 On the other hand,
morphological LA is connected to morphological RV on
left side, which gives rise to aorta (Figs 72.150 and 72.151).
This anomaly can be encountered with situs solitus or
with situs inversus. Anomalies having univentricular AV
connection such as atresia of one of the AV valves, and
double inlet LV with hypoplastic RV on left side should be
excluded.
The aorta usually arises left and anterior to pulmonary
valve (L-transposed aorta), but rarely aorta can be right
and anterior to pulmonary trunk. There will be fibrous
continuity between mitral leaflet and the pulmonary valve.
On the other side, aorta is separated from tricuspid valve
by the muscular infundibulum.
Fig. 72.150: Two-dimensional echocardiography. Subcostal coronal view in a patient of corrected transposition of great vessels
(CTGA) showing atrioventricular discordance. (LA: Left atrium; LV:
Left ventricle; PA: Pulmonary artery; RA: Right atrium; RV: Right
ventricle).
Associated Defects221232
The commonest associated anomaly is left AV valve
(tricuspid valve) abnormalities.220 The other lesions are
VSD and pulmonary stenosis. However, any kind of
congenital cardiac malformation can be encountered.
Fig. 72.151: Two-dimensional echocardiography. Subcostal coronal view with anterior tilt in a child of corrected transposition of
great vessels (CTGA) and ventricular septal defect (VSD; star)
showing the L-looped ventricles with aorta arising from morphological left-sided right ventricle and pulmonary artery arising from
morphological left ventricle (right-sided). Mitral and pulmonary
valves show continuity (arrow). (Ao: Aorta; LV: Left ventricle; PA:
Pulmonary artery; RV: Right ventricle).
1665
Fig. 72.153: Two-dimensional echocardiography. Apical fourchamber view from a 4-year-old child with corrected transposition
of great arteries showing atrioventricular discordance, reverse
offsetting of atrioventricular (AV) valves (arrow), and moderator
band in morphological right ventricle on left side (arrow). (LA: Left
atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
1666
Fig. 72.155: Two-dimensional echocardiography with fourchamber view with anterior tilt in a case of corrected transposition
of great vessels (CTGA) showing a regressed left ventricle; there is
no left ventricular (LV) outflow tract obstruction. (LV: Left ventricle;
PA: Pulmonary artery; RV: Right ventricle).
Figs 72.156A and B: Two-dimensional echocardiography. Apical four-chamber view in a case of corrected transposition of great vessels
(CTGA) showing the comparison of the normal heart with normal offsetting (arrow) (Figure A) of atrioventricular valves as compared to
the CTGA with reverse offsetting and the trabeculated right ventricle (RV) with a moderator band (Figure B). (LA: Left atrium; LV: Left
ventricle; RA: Right atrium; RV: Right ventricle).
Associated Malformations
Ventricular Septal Defect
It is reported in 6070% of patients with CTGA. The
commonest is perimembranous defect (Fig. 72.151), which
is subpulmonary and extends posteriorly toward the crux of
the heart and erodes significantly the inlet septum. Fibrous
continuity between mitral, tricuspid, and pulmonary valve
is present. It is important to define VSD relation to AV valves
and document if there is any overriding or straddling of AV
valves, more commonly of tricuspid valve. In cases with
1667
Fig. 72.157: Two-dimensional echocardiography. Modified parasternal long-axis view showing the origin of the pulmonary artery
from the left ventricle (LV) with prolapse of the mitral valve into
the left ventricular outflow tract (LVOT; arrow) causing obstruction.
(LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).
1668
Figs 72.158A and B: Two-dimensional echocardiography. Apical four-chamber view with color compare with anterior tilt showing the
origin of the pulmonary artery from the left ventricle and the presence of supravalvular pulmonary stenosis (white arrow). A ventricular
septal defect is noted (yellow arrow) in the anterior muscular plane. (LV: Left ventricle; PA: Pulmonary artery; RV: Right ventricle).
Fig. 72.159: Two-dimensional echocardiography with color comparison showing a small anterior muscular ventricular septal
defect (arrow) in a case of corrected transposition of great vessels
(CTGA). (LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery;
RV: Right ventricle; RA: Right atrium).
Fig. 72.160: Two-dimensional echocardiography. Apical fourchamber view with color flow mapping showing severe tricuspid
regurgitation (TR) in Ebsteins anomaly of the left-sided atrioventricular (AV) valve in a case of corrected transposition of great vessels (CTGA). (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).
to supratricuspid ring or membrane. From apical fourchamber and PLAX view, this membrane is seen as thin,
linear echoes just above the tricuspid valve, attaching
laterally to free wall of LA and medially to left atrial
surface just above the crux. Color flow mapping shows
turbulence beginning above the level of valve, and on
pulsed or continuous wave Doppler interrogation, severity
of obstruction can be defined. Rarely cor triatriatum
sinister may be the cause of tricuspid inflow obstruction.
Best views to profile cor triatriatum are PLAX and fourchamber views. In PLAX view, the membrane extends
1669
Fig. 72.161: Two-dimensional echocardiography in apical fourchamber view in a case of corrected transposition of great vessels
(CTGA) showing Grade I straddling of the left atrioventricular
valve (tricuspid) across the large inlet ventricular septal defect
(arrow). (LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV:
Right ventricle).
1670
Fig. 72.164: Two-dimensional echocardiography in apical fourchamber view with anterior tilt showing a pulmonary artery (PA)
band in situ with flow acceleration across it (arrow). (LV: Left
ventricle; RV: Right ventricle; PA: Pulmonary artery).
1671
Figs 72.165A and B: Two-dimensional echocardiography in subcostal coronal view with color flow mapping showing right upper and
left upper pulmonary veins draining into left atrium (LA; arrows). LA: Left atrium; RA: Right atrium).
Figs 72.166A and B: Two-dimensional echocardiography in sagittal subcostal view showing right upper and right lower pulmonary veins
draining to left atrium (LA; arrows). (LA: Left atrium; RA: Right atrium).
1672
Fig. 72.170: Two-dimensional echocardiography. Apical fourchamber view in a case of total anomalous pulmonary venous
connection (TAPVC) showing markedly dilated right atrium and
right ventricle. (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).
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Figs 72.172A and B: Suprasternal short-axis view with color flow mapping in a case of supracardiac total anomalous pulmonary venous
connection (TAPVC). (A) Shows right- and left-sided pulmonary veins forming a pulmonary venous confluence; (B) Shows pulmonary
venous confluence continuing as vertical vein. (PVC: Pulmonary venous confluence; RPA: Right pulmonary artery; VV: Vertical vein).
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Figs 72.178A and B: (A) Two-dimensional echocardiography with color flow mapping. Modified suprasternal short-axis view showing
turbulence in the ascending vertical vein in a case of supracardiac total anomalous pulmonary venous connection; (B) Continuous
wave Doppler tracing showing the continuous flow throughout the cardiac cycle with a mean gradient of 5 mm Hg. (Ao: Aorta; Innom:
Innominate vein; VV: Vertical vein).
1677
Figs 72.180A and B: Two-dimensional echocardiography. Subcostal coronal view with color compare in a case of total anomalous
pulmonary venous drainage showing a malaligned interatrial septum (arrow) resulting in the drainage of the pulmonary veins occurs into
the right atrium (arrows). (LA: Left atrium; RA: Right atrium).
Fig. 72.181: Two-dimensional echocardiography. Apical fourchamber view showing malaligned interatrial septum (arrow) leading to total anomalous pulmonary venous drainage with normally
connected pulmonary veins. RA is markedly dilated. (LA: Left
atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
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Figs 72.183A and B: Two-dimensional echocardiography in suprasternal short-axis view with color compare showing a large left
superior vena cava (SVC) draining to coronary sinus. (CS: Coronary sinus; LSVC: Left superior vena cava).
Clinical Significance
Sinoatrial junction is poorly developed in these cases and
patients, usually adults, may develop sick sinus syndrome
requiring a pacemaker. Issues that make the diagnosis of
absent right SVC important include: (a) implantation of
transvenous pacemaker, (b) placement of transvenous
pulmonary catheter for monitoring particularly without
the usage of fluoroscopy, (c) systemic venous cannulation
for extracorporeal membrane oxygenation (ECMO),
(d) systemic venous cannulation for cardiopulmonary
bypass, (e) partial or total cavopulmonary anastomosis,
(f ) orthotopic heart transplantation, (g) endomyocardial
biopsies, and (h) cardiac catheterization particularly for
interventricular procedures.
Special precaution should be taken at the time of openheart surgery to avoid damage to area around coronary
sinus, and ligation of coronary sinus must be avoided.
Echocardiography: Suprasternal short-axis view is
especially important for determining whether right SVC is
present or not, in addition to profiling of LSVC to coronary
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Figs 72.184A and B: Two-dimensional echocardiography. Subcostal sagittal view with color compare showing the drainage of right
superior vena cava (SVC; arrow) to left atrium. (LA: Left atrium; RA: Right atrium; SVC: Superior vena cava).
Fig. 72.185: Saline contrast study. Saline was injected into the
right brachial vein and contrast echoes appeared in the left atrium
followed by left ventricle. No contrast appeared in the right atrium
and right ventricle, clearly showing communication of superior
vena cava (SVC) to left atrium. (LA: Left atrium; LV: Left ventricle;
RA: Right atrium; RV: Right ventricle; SVC: Superior vena cava).
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Figs 72.188A to D: Two-dimensional echocardiography with color flow mapping showing normal coronaries. (A) Parasternal short-axis
view showing the right coronary artery (RCA) and the left anterior descending artery (LAD, arrow); (B) Color flow mapping showing the
course of LAD; (C) Parasternal short-axis view showing the origin of the right coronary artery (arrow); (D) Color flow mapping showing
right coronary artery (RCA). (Ao: Aorta; PA: Pulmonary artery; RVOT: Right ventricular outflow tract).
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Figs 72.190A and B: Two-dimensional echocardiography. (A) Shows the origin of the left main coronary artery (marked by the arrow)
from the pulmonary artery; (B) Color flow mapping shows flow reversal in the coronary artery with left anterior descending artery (LAD)
showing blue color flow signals indicative of flow toward the pulmonary artery in a case of anomalous origin of left coronary artery from
pulmonary artery (ALCAPA). (Ao: Aorta; PA: Pulmonary artery).
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Figs 72.191A and B: Two-dimensional echocardiography. Apical four-chamber view showing the sclerosed papillary muscle (arrow)
in a case of anomalous left coronary artery from pulmonary artery; (B) Color flow mapping showing mild mitral regurgitation. (LA: Left
atrium; LV: Left ventricle).
Fig. 72.192: Two-dimensional echocardiography. Modified parasternal short-axis view with color flow mapping in a case of
anomalous left coronary from pulmonary artery showing a dilated
right coronary artery (RCA; arrow) with multiple collaterals from
right coronary artery (arrow). (Ao: Aorta).
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CORONARY ARTERIOVENOUS
FISTULA (FIGS 72.194A TO C)
Coronary arteriovenous fistulas are present in 1 of 50,000
live births (0.002% of the general population) and are
visualized in 1 of 500 patients undergoing catheterization
(0.20.25%).288,289 The etiology of these lesions may be
congenital or acquired; the latter may be broken down
into infectious, traumatic, and iatrogenic. Iatrogenic
causes may be further subdivided as after surgery,
catheterization, angioplasty, or endomyocardial biopsy.
We will be discussing congenital coronary arteriovenous
fistulae only.
Coronary arteriovenous fistula involves RCA in 60%,
LAD artery in 25%, and left circumflex artery in 15% of
patients. Rarely it involves more than one coronary artery.
CORONARY ANEURYSMS
Aneurysms are defined as dilations of a coronary vessel
1.5 times the adjacent normal coronaries. The aneurysm
can be saccular or fusiform, fusiform being the most
common. Aneurysms may be congenital or acquired,
the latter may be further subdivided into atherosclerotic,
Kawasaki disease, traumatic, iatrogenic (surgical, after
angioplasty, catheterization, or endomyocardial biopsy),
infectious, and systemic diseases (polyarteritis nodosa,
syphilis, EhlersDanlos syndrome, Marfan disease, and
scleroderma).
Kawasaki Disease
Kawasaki disease is the commonest disease associated
with coronary aneurysms in the pediatric age group.
Moderate coronary involvement with aneurysms is present
in 12.8 to 25% of patients with untreated Kawasaki disease;
the incidence of coronary involvement reduces to one
fifth after timely intravenous gamma globulin therapy. The
following definitions pertaining to Kawasaki disease are
generally accepted in the literature: segmental stenosisa
braid-like lesion with multiple tortuosities of the vessel,
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Vascular Rings
1692
of coarctation anatomy in most patients. High quality ultrasound images can be obtained in infants but may be somewhat difficult to obtain in larger children and adolescents.
Echocardiographic objectives:
Is there coarctation?
Is there discrete stenosis or long segment stenosis?
Is there arch hypoplasia?
Branching pattern of arch.
Are there associated cardiac anomalies, in particular,
bicuspid aortic valve, left-sided obstructive lesions
such as aortic stenosis, subaortic membrane, mitral
valve disease, or hypoplastic left heart?
Left ventricular hypertrophy.
Ventricular function.
The echocardiographic examination using the suprasternal long-axis view provides an image of the entire arch,
with the area of coarctation seen near the origin of left
subclavian artery, that is, juxtaductal CoA (Figs 72.200A
and B). On 2D echocardiography, it is seen as a prominent
posterior shelf with significant coarctation. When imaging
the arch, one must be absolutely certain that the entire arch
is imaged, particularly in the region of the left subclavian
artery. The diagnosis can be missed with inadequate
imaging in this area.
Type of coarctation may either be a long segment
narrowing or, more commonly short segment obstruction
caused by posterior endothelial shelf projecting into
the aorta (Figs 72.197A and B). There may be associated
Figs 72.197A and B: Two-dimensional echocardiography with color compare. (A) Suprasternal long-axis view with color compare in a
child of coarctation of aorta showing tiny posterior (arrow) shelf with flow acceleration; (B) Suprasternal long-axis view with color compare in another case of coarctation of aorta showing a prominent posterior shelf (arrow) with significant turbulence in the arch starting
from the coarctation region. (As Ao: Ascending aorta; Ds Ao: Descending aorta; TA: Transverse arch).
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Figs 72.199A and B: Continuous wave Doppler signals across the coarctation of aorta. (A) Demonstrates mild coarctation of aorta
with early diastolic spill and a gradient of 31 mm Hg; (B) Shows severe coarctation of aorta with pan-diastolic flow and a gradient of
61 mm Hg.
Figs 72.200A and B: Two-dimensional echocardiography. Suprasternal long-axis view showing interruption of the aortic arch distal to
the left subclavian artery with arch hypoplasia (arrow); (B) Represents suprasternal long-axis view showing interruption of the arch of
aorta (arrow) between the carotid arteries (type C). (As Ao: Ascending aorta; Ds Ao: Descending aorta; TA: Transverse arch).
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With retroesophageal subclavian artery (i.e. both carotid arteries proximal, both subclavians distal)
Descending aorta is relatively dilated with ductal continuation to descending aorta, which is well profiled in
suprasternal long-axis and high PSAX views. Care should
be taken to ensure that the ductus arteriosus connecting the
main PA to descending aorta (ductal arch) is not mistaken
for the true arch. Color flow mapping confirms the findings
and as systemic flow is duct-dependant, patency of duct
or any restriction of duct should be defined by color flow
mapping. Pulsed Doppler is used to assess the gradient
across the ductus. Sometimes, restriction of ductus
arteriosus can mimic CoA, and that should be clearly
defined. In patients with suprasystemic PA pressures, there
can be turbulent right to left flow across the ductus.
AORTIC ANEURYSM
The third important subgroup of aortic arch anomalies
includes aneurysms of the aorta. Aneurysm of a vessel
is defined as a dilated segment > 50% in diameter as
compared to the proximal segment. In pediatric age
group, in contrast to adults, aneurysm of ascending aorta
is more common than descending aorta. Annuloaortic
ectasia is defined as aneurysmal involvement of annulus
and aortic root, in addition to ascending aorta. In children,
aneurysmal dilatation of proximal aorta can be caused by
conditions associated with medial degeneration of the
aorta such as Marfan syndrome, EhlersDanlos syndrome,
Turner syndrome, in association with bicuspid aortic valve,
and idiopathic; however, some types of infectious disease
like bacterial endocarditis can also result in aneurysm
formation. Aneurysm of proximal aorta is usually easily
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Fig. 72.202: Two-dimensional echocardiography. Apical fourchamber view showing the two inlets into a ventricle with left ventricular morphology in a case of double inlet left ventricle (DILV).
(LA: Left atrium; LV: Left ventricle; RA: Right atrium).
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Fig. 72.203: Two-dimensional echocardiography. Apical fourchamber view showing in a case of tricuspid atresia with hypoplastic right ventricle (RV), a large atrial septal defect (arrow) and
a small muscular ventricular septal defect (VSD; star). (LA: Left
atrium; LV: Left ventricle; RV: Right ventricle; RA: Right atrium).
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Figs 72.204A and B: Two-dimensional echocardiography. (A) Apical four-chamber view showing complete atrioventricular septal defect
(AVSD) with a nonapex-forming small left ventricular (LV) cavity not suitable for biventricular pathway; (B) Shows a common AVSD
(unbalanced type) with hypoplastic right ventricle (RV) on a single ventricular pathway. (LA: Left atrium; LV: Left ventricle; RA: Right
atrium; RV: Right ventricle).
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Bulboventricular Foramen
The bulboventricular foramen is the orifice through
which the main ventricular chamber feeds blood to the
rudimentary outflow chamber. If the chamber supports
the pulmonary flows like in Holmes heart, then restriction
of the bulboventricular foramen results in restricted
pulmonary blood flow. If the rudimentary outflow
chamber supports the aortic circulation, restriction of
bulboventricular foramen results in subaortic obstruction.
To calculate the area of bulboventricular foramen, measure
the maximum diameter of the bulboventricular foramen
in two orthogonal planes. If the dimensions are a and b,
then the area of the bulboventricular foramen is calculated
assuming it to be an ellipse using the formula:
Area = a + b/4
When the area of the bulboventricular foramen is
> 2.0 cm2/m2, the foramen is considered nonrestrictive.
In patients with area < 2.0 cm2/m2, during the initial
palliation, the restrictive bulboventricular foramen may
need to be addressed using one of the surgical strategies
like DamusKayeStansel procedure (end-to-side anastomosis of the main PA to the ascending aorta) or
enlargement of the bulboventricular foramen. In patients
in whom the bulboventricular foramen is anatomically
smaller though nonrestrictive by Doppler recordings, a
close echocardiographic watch is justified. The progression
of the hemodynamic narrowing of the bulboventricular
foramen is explained as follows: initially with acute volume
unloading of the ventricle after PA banding, there is an
acute reduction of the ventricular volumes and reduction
of the bulboventricular foramen area. Over long-term
follow-up, there is progressive ventricular hypertrophy,
which too results in further restriction of bulboventricular
foramen size. In neonates who are too young and small to
tolerate an enlargement of bulboventricular foramen or
DamusKayeStansel procedure, alternative approaches
followed include palliative arterial switch operation to
shift the restrictive rudimentary outflow chamber from
subaortic location to subpulmonary location. Alternative
method is the measurement of the VSD size and comparing
it with the aortic annulus size. If the 2D measurement of
the VSD is less than aorta, it requires enlargement.
TRICUSPID ATRESIA
Tricuspid atresia is defined as complete agenesis of the
tricuspid valve with no direct communication between the
RA and RV. Morphologically, this entity is classified as in
Table 72.17.
Echocardiographic differentiation between tricuspid
atresia and single ventricle with right AV valve atresia has
already been highlighted in the previous discussion. We
will now discuss the important echocardiographic features
of this entity.
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the duct, since the arch and ascending aorta get retrograde
flows from the patent ductus. Prompt recognition of
such flow reversal in aortic arch and duct dependence
of systemic circulation will help in early institution of
prostaglandin E1 therapy and transfer to an advanced
cardiac surgical center.
Another important echocardiographic decision-making issue is identification of adequacy of interatrial septal
communication. Since ASD is the only outlet for pulmonary venous blood, inadequate atrial communication will
need urgent enlargement either in cardiac catheterization
suites or operation rooms. In some cases of mitral atresia,
decompression of the LA through a small unroofing of the
coronary sinus may be present.
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Figs 72.205A to D: Two-dimensional echocardiography with color flow mapping in a case of left ventricular (LV) hypoplasia. (A) Fourchamber view with color flow mapping showing a restrictive interatrial communication; (B and C) Apical four-chamber view with color
flow compare showing a small LV cavity with thickened endocardium and flow acceleration across the mitral valve; (D) Suprasternal
view showing a hypoplastic ascending aorta and transverse arch. (As Ao: Ascending aorta; Ds Ao: Descending aorta; LA: Left atrium;
LV: Left ventricle; RA: Right atrium; TA: Transverse arch).
Subcostal View
As the transducer is tilted toward the cardiac base, the
coronary sinus, RA, and RV are visualized. A dilated
coronary sinus should alert one for the presence of
LSVC or anomalous pulmonary venous connection to
the coronary sinus. The RA in typical scenario is dilated
and hypertrophied from obligatory left-to-right shunting
through the patent foramen ovale. The RV also is dilated
Apical Views
Apical four-chamber view provides comparison of the
relative right and left sides of the heart. The RV is generally
large and hypertrophied, and the LV is small, musclebound, and nonapex forming. The endocardial surface
of the LV is often echogenic because of endocardial
fibroelastosis. This in itself is a very important prognostic
marker (particularly in borderline situations where one is
contemplating a two-ventricle repair). The morphology of
the mitral and the tricuspid valve can also be evaluated
in this view. The annulus of the mitral valve is typically
hypoplastic, severely stenosed, or may be atretic. This
may be evaluated using color flow mapping and Doppler
assessment. In case of stenosis of the mitral valve without
mitral regurgitation, one should be alerted to the likelihood
of ventriculocoronary sinusoids.
Tricuspid valve morphology and function needs to be
assessed also. It is important to identify and quantify the
tricuspid regurgitation, an important prognostic feature.
RV function is also assessed in this view.
Anterior tilt will show the aortic valve, ascending aorta,
and pulmonary trunk. Doppler assessment of the aortic
valve and color flow mapping will show the patency of the
aortic valve. The pulmonary valve should also be similarly
investigated.
Parasternal Views
Parasternal views are important to assess ventricular
function and size and function of the AV valves. The
dilated RV is seen anteriorly and the small LV, posteriorly.
The ventricular septum is usually intact. The aortic valve
is either stenosed or atretic and LVOT may have subaortic
obstruction, which can also be seen in this view. The
morphology of the mitral valve should be studied and
the dimensions of the mitral and aortic valve annulus
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Echocardiographic Assessment
After the Stage 1 Palliation
The Norwood procedure consists of surgical
reconstruction and augmentation of the ascending aorta
and aortic arch with aortopulmonary amalgamation,
an atrial septectomy, and systemic to pulmonary shunt,
which can be either a BlalockTaussig shunt or a RV to PA
conduit (Sanos modification and PA banding). Interatrial
septal assessment is important as restriction may occur
mostly due to inadequate resection, an important cause
of cyanosis. The right BT shunt may be visualized from
the suprasternal short- or long-axis views with angulation
of the transducer toward the right side. Postoperative
complications of the shunt including pulmonary over
circulation, shunt stenosis, and hence PA distortion
may occur and need evaluation. Sanos shunt can be
best visualized by subcostal and modified apical views.
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Anatomical Background:
Characterization of Two Distinct
Heterotaxy Syndromes
Two distinct patterns emerge when we consider cardiac
abnormalities in these complex syndromesright
isomerism and left isomerism. Right isomerism is
1705
Right Isomerism
Left Isomerism
Dextrocardia
40%
40%
70%
10%
Rare
60%
50%
40%
Rare
70%
70%
Rare
Rare
40%
90%
80%
85%
40%
Single ventricle
50%
10%
80%
30%
PS/pulmonary atresia
80%
30%
LVOTO
Rare
40
Extracardiac anomalies
CNS, gastrointestinal,
skeletal and genitourinary
Biliary atresia
Intestinal malrotation
Agenesis of gall bladder
Source: Gutgesell HP. Cardiac Malposition and Heterotaxy. In: Garson Jr, Bricker JT, Fischer DJ, Neish SR, editors. The Science and
Practice of Pediatric Cardiology. Baltimore, MD: Williams & Wilkins; 1998:153961.
1706
Right Isomerism
Left Isomerism
29%
22%
Unilateral to left
20%
16%
Bilateral to roof
51%
38%
24%
To right-sided atrium
48%
12%
To left-sided atrium
52%
12%
Interrupted on right
34%
Interrupted on left
42%
Hepatic veins
Confluence to IVC
76%
14%
Confluence to atrium
43%
Unilateral to atrium
6%
8%
Bilateral to atrium
18%
35%
To right-sided atrium
19%
26%
To left-sided atrium
19%
14%
Bilaterally to atriums
60%
3%
To extracardiac site
59%
Pulmonary veins
Table 72.20: The Echocardiographic Checklist for the Univentricular Pathway Patient
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Atrial Septum
Common atrium with both atria having right morphology
is often associated with right isomerism. In patients
with left isomerism, 50% of cases have common atrium.
The morphology of the atrial septum does not help in
differentiating between right and left isomerism. The
ASD is best imaged using the subcostal coronal and
sagittal views. The adequacy of the size of the interatrial
communication is important before consideration of a
single ventricle repair.
Atrioventricular Junction
The atrioventricular connections in patients with
isomerism can be biventricular or univentricular. The
ventricular topology can be either D-loop or L-loop,
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Ventriculoarterial Connections
In patients with right isomerism, the common abnormalities of the ventriculoarterial connections are DORV
with pulmonic stenosis/atresia and single outlet with no
pulmonary outflow. Discordant ventriculoarterial connections may also be seen. The assessment of severity of
pulmonary stenosis is very important. The outflow tracts
can be imaged using subcostal coronal, four-chamber,
and PSAX views. Doppler evaluation of the gradient
across the pulmonary outflow (subvalvular and valve)
will provide an estimate of PA pressures. However, in
presence of pulmonary venous obstruction, Doppler
estimation of the gradient may underestimate the severity
of the obstruction of outflow. In such cases, attention
should focus on morphology of the outflow tract. In left
isomerism, concordant ventriculoarterial connections are
more common and obstruction to pulmonary outflow is
less common.
ACKNOWLEDGMENTS
We are indebted to our colleagues for their cooperation.
We wish to express our heartfelt thanks and gratitude to
our secretary Ms Poonam Toppo for her secretarial help
and Dr Kunal Bhagatwala for the correction of the proof of
the chapter and helping with the movie clips.
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1720
CHAPTER 73
Real Time 3D Echocardiography for
Quantification of Ventricular Volumes,
Mass and Function in Children with
Congenital and Acquired
Heart Diseases
Shuping Ge, Jie Sun, Lindsay Rogers, Rula Balluz
Snapshot
INTRODUCTION
The advent of real time three-dimensional echocar
diography (RT3DE) is a major milestone in the evolution
of echocardiography. RT3DE has been used to generate
three-dimensional (3D) views of cardiac structures in an
intuitive and object format, obviating the need to mentally
create 3D images based on sweeps by two-dimensional
echocardiography (2DE). Furthermore, RT3DE provides
an opportunity to quantitatively measure distances, areas,
volumes to quantify the cardiac structures, flows, and
function in 3D that may not possible by 2DE. A query of
3D echocardiography yields over 4,500 publications
in PubMed database. Therefore, the focus of this chapter
is the use of RT3DE to quantitatively assess ventricular
volumes, mass, and function in children with congenital
and acquired heart diseases. Other related topics can be
found in other chapters of this book.
Future Perspectives
1722
Chapter 73: Real Time 3D Echocardiography for Quantification of Ventricular Volumes, Mass and Function in Children
Meta-Analysis
Shimada and Shiota39 conducted a meta-analysis to assess
the sources of errors in evaluation of the LV by 3DE. Their
analysis included 3,055 subjects in 95 studies. The study
showed significant underestimation bias of both LVEDV
and LVESV by RT3DE compared with CMR. The bias
for estimation of LVEF was not statistically significant.
Sources of error included female sex and presence of
congenital heart disease, which were associated with
more underestimation in the analysis. Semiautomatic
border detection and the use of matrix-array transducer
were associated with less underestimation. Despite the
differences between RT3DE volumetric estimation of the
LV and CMR, the literature supports the role of RT3DE as
both accurate and reproducible in assessing LV volume
and LVEF, although it is not interchangeable with other
radiological modalities.
Shimada and Shiota40 published another meta-analysis
evaluating the use of RT3DE to assess LV mass. This analysis
assessed 25 studies including 671 comparisons. Studies
published in 2004 or earlier showed high heterogeneity
[I(2) = 69%)] and significant underestimation of LV mass
by 3DE (5.7 g, 95% confidence interval 11.3 to 0.2,
P = 0.04). Studies published between 2005 and 2007
were still heterogeneous [(I(2) = 60%)] but showed less
systematic bias (0.5 g, 95% confidence interval 2.5 to 1.5,
P = 0.63). In contrast, studies published in 2008 or later were
highly homogeneous [(I(2) = 3%)] and showed excellent
accuracy (0.1 g, 95% confidence interval 2.2 to 1.9,
P = 0.90). Investigation of factors affecting the bias revealed
that evaluation of cardiac patients compared with healthy
volunteers led to greater bias (P < 0.05). In conclusion,
this meta-analysis elucidates the underestimation of LV
mass by 3DE, improvement of the technique over the past
decade, and factors affecting the degree of bias. These data
1723
1724
Table 73.1: Real Time Three-Dimensional Echocardiography for Left Ventricle Volumes, Mass, and Function in Children
Methods
LV Indices
Correlation
19
CMR
Long-axis, eight
planes
LVEDV, LVESV,
LV mass, LVEF,
SV
r = 0.860.97
2007
25
LV angiography
Semiautomated
LVEDV and
LVES
r = 0.9790.996
Lu et al.6
2008
20
CMR
LVEDV, LVESV,
LV mass, LVEF,
SV
r = 0.850.9
Riehle et al.7
2008
12
CMR
Semiautomated
LVEDV, LVESV,
LV mass, LVEF,
SV
Friedberg
et al.8
2010
35
CMR
Disc summation
LVEDV, LVESV,
LVEF
r = 0.900.96
Hascot et al.9
2010
50
RT3DE
Semiautomated
(Qlab vs. TomTec)
LVEDV, LVESV,
LV mass, LVEF,
SV
Laser et al.10
2010
49
CMR
2 Ultrasound systems/
semiautomated
LVEDV and
LVESV
r = 0.910.95
Reference
Year
Number of
Patients
Bu et al.4
2005
Iino et al.5
(CMR: Cardiac magnetic resonance imaging; EF: Ejection fraction; LV: Left ventricle; LVEDV: Left ventricular end-diastolic volume
LVEF: Left ventricular ejection fraction; LVESV: Left ventricular end-systolic volume; RT3DE: Real time three-dimensional echocardiography; SV: Stroke volume).
Chapter 73: Real Time 3D Echocardiography for Quantification of Ventricular Volumes, Mass and Function in Children
1725
Figs 73.2A to C: (A) The disc summation method for measurement of right ventricular (RV) volumes and ejection fraction (EF)
in children. Manual tracing of the RV endocardial contours was
performed on a stack of short-axis images. The four-chamber
views and coronal views are used as reference images; (B) Simpsons principle is used to derive RVEDV, RVESV, and right ventricular ejection fraction (RVEF); (C) The semiautomated method
for measurement of the RV indices. The method is based on the
Beutel model with manual tracing of the RV endocardial land
marks, including a set of coronal, sagittal, and four-chamber views.
(LV: Left ventricle; RV: Right ventricle).
Source: Adapted from Ref. 96.
Meta-Analysis
Shimada et al.60 performed a meta-analysis of published
studies comparing assessment of RV volumes and EF by
RT3DE versus CMR. The analysis included 23 studies and
807 adults and children. They concluded that compared
with CMR, 3DE significantly underestimated RVESV,
1726
THREE-DIMENSIONAL ANALYSIS
OF REGIONAL WALL MOTION,
SYNCHRONY, AND STRAIN
Regional Wall Motion and Synchrony
The capability of RT3DE to capture the entire LV in three
dimensions offers the opportunity not only to assess
Chapter 73: Real Time 3D Echocardiography for Quantification of Ventricular Volumes, Mass and Function in Children
1727
Figs 73.3A and B: Three-dimensional speckle tracking echocardiography (3DSTE) offline analysis. The endocardial borders were
manually traced at left ventricle (LV) end-diastole and end-systole from the apical four-chamber, two-chamber, and LV long-axis views,
respectively. The LVEDV, LVESV, left ventricular ejection fraction (LVEF), systolic dyssynchrony index (SDI), LV three-dimensional (3D)
systolic peak global (A) and segmental strain (B) were automatically calculated by the software. The mean systolic 3D peak strain of the
16 segments represented LV global strain. The mean basal, mid, and apical segmental systolic 3D peak strain represented the LV segmental peak systolic strain, respectively. The 3D strain indices derived included global systolic strain (GSS); global longitudinal systolic
strain (GLSS); systolic strain of basal, middle, and apical segments.
Source: Adapted from Ref. 96.
1728
FUTURE PERSPECTIVES
Much evidence suggests that in the presence of adequate
image quality, LV volumes, mass, and EF measurements
by 3DE agree more closely with CMR measurements
and have better reproducibility than does 2DE, making
3DE the modality of choice for the everyday clinical
evaluation of LV volumes and EF. Quantitative analysis
of volumes, mass, and EF for the RV and for single
ventricles holds great promise and warrants future
investigation. New modes of regional wall motion, strain,
and LV dyssynchrony assessment using RT3DE are in
the forefront of active research. Future advancements in
hardware are expected to allow acquisition of wide-angle
3D data from the entire heart in a single cardiac cycle,
with higher spatial and temporal resolution. We anticipate
that further development in automatic quantitative
analysis algorithms and software will make RT3DE an
integral clinical methodology for diagnosis, prognosis,
and assessment of medical, percutaneous, and surgical
intervention for congenital heart disease.87
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ventricular volume and mass measurement by real-time
three-dimensional echocardiography. Echocardiography.
2006;23(5):3959.
46. Hoch M, Vasilyev NV, Soriano B, Gauvreau K, Marx GR.
Variables influencing the accuracy of right ventricular
volume assessment by real-time 3-dimensional
echocardiography: an in vitro validation study. J Am Soc
Echocardiogr. 2007;20(5):45661.
47. Liu YN, Deng YB, Liu BB, et al. Rapid and accurate quanti
fication of right ventricular volume and stroke volume by
real-time 3-dimensional triplane echocardiography. Clin
Cardiol. 2008;31(8):37882.
48. Kjaergaard J, Petersen CL, Kjaer A, et al. Evaluation of
right ventricular volume and function by 2D and 3D
echocardiography compared to MRI. Eur J Echocardiogr.
2006;7(6):4308.
49. Nesser HJ, Tkalec W, Patel AR, et al. Quantitation of
right ventricular volumes and ejection fraction by threedimensional echocardiography in patients: comparison
with magnetic resonance imaging and radionuclide
ventriculography. Echocardiography. 2006;23(8):66680.
50. Gopal AS, Chukwu EO, Iwuchukwu CJ, et al. Normal
values of right ventricular size and function by real-time
3-dimensional echocardiography: comparison with cardiac
magnetic resonance imaging. J Am Soc Echocardiogr.
2007;20(5):44555.
51. Jenkins C, Chan J, Bricknell K, et al. Reproducibility of right
ventricular volumes and ejection fraction using real-time
three-dimensional echo
cardiography: comparison with
cardiac MRI. Chest. 2007;131(6):184451.
52. Sugeng L, Mor-Avi V, Weinert L, et al. Multimodality
comparison of quantitative volumetric analysis of the right
ventricle. JACC Cardiovasc Imaging. 2010;3(1):1018.
53. Leibundgut G, Rohner A, Grize L, et al. Dynamic assessment
of right ventricular volumes and function by real-time
three-dimensional echocardiography: a comparison study
with magnetic resonance imaging in 100 adult patients.
J Am Soc Echocardiogr. 2010;23(2):11626.
Chapter 73: Real Time 3D Echocardiography for Quantification of Ventricular Volumes, Mass and Function in Children
1731
1732
CHAPTER 74
Three-Dimensional Echocardiography
in Congenital Heart Disease
Steven Bleich, Gerald R Marx, Navin C Nanda, Fadi G Hage
Snapshot
Common Atrium
Aortopulmonary Window
Conotruncal Anomalies
INTRODUCTION
Congenital heart disease (CHD) includes an extensive
group of diagnoses affecting both pediatric and
adult patients. The complexity of CHD has motivated
cardiologists to better understand each individual defect
in order to maximize management of these patients
and improve treatment options. Two-dimensional (2D)
echocardiography has been the gold standard imaging
modality used because of its convenience, low cost, and lack
of harmful side effects as compared to magnetic resonance
imaging (MRI) and multidetector computed tomography
(CT).1,2 However, identifying and understanding CHD
often requires a real life, three-dimensional (3D) image
of the defect.35 With 2D echocardiography, the reader
often expends effort to mentally reconstruct the heart and
great vessels in three dimensions, sometimes, preventing
visualization of relevant details. The emergence of 3D
echocardiography (3DE) provides a more comprehensive
view of the cardiac anatomy which offers incremental
value in the diagnosis of CHD.
3DE has gained popularity in the field of CHD because
it can portray a dynamic and realistic picture of the
Other Abnormalies
1734
Secundum ASD
Secundum ASDs and patent foramen ovale (PFO) occupy
the middle portion of the atrial septum. Although 2D
echocardiography has historically been the imaging
modality of choice in diagnosing these ASDs, images
obtained via this method can lack optimal viewing of the
defect and therefore distort the measurements obtained.15
In the setting of multiple fenestrations such as the Swiss
cheese pattern or multiple hole pattern, the limitations of
2D TTE are even more obvious. The key to any successful
echocardiographic image relies on a clear imaging
window with the patient in a position that provides the
best view for the reader. 3D TTE performed using the
apical, para-apical, right parasternal and subcostal views
provides adequate en face visualization of the defect in
most patients (Figs 74.2 and 74.3). The interatrial septum
is often best observed in the right parasternal view with
the patient in the right lateral decubitus position.15
Information regarding exact dimensions and location of
an ASD along with relative surrounding cardiac anatomy
and rim size can be obtained more easily by 3D TTE than
2D transesophageal echocardiography (TEE).15,16 With
the advent of real time 3D TTE, a PFO can be visualized
as well as the motion of the valve covering it (Figs 74.4
and 74.5).17 Recognizing the development of worsening
dyspnea and hypoxia with change in position from the
supine to sitting position suggests the possibility of
platypnea-orthodeoxia syndrome. 3D TEE has been used
to document an open PFO with shunting of blood in the
sitting position and closed PFO in the supine position
(Figs 74.6 and 74.7).18 One study done by Morgan et al.
compared the results obtained by 2D TEE to 3D TTE
with respect to maximum defect diameter, area and
circumference of an ASD.19 Data obtained from these two
modalities were not statistically different, however, 3D
TTE compared to 2D TEE provided clinically significantly
information. 3D was able to recognize appropriate
candidates for percutaneous closure of an ASD while
eliminating the need for more invasive imaging with TEE.
Repair of ASDs have traditionally been performed
with surgery, however, recent evidence recommends the
use of more percutaneous closure devices. The decision
to proceed with surgery versus percutaneous approach
depends on the location of the ASD, its shape and size,
and the surrounding tissue.4,15 In order to proceed with
percutaneous repair, the cutoff with regard to rim size has
1735
Figs 74.1A to D: Live three-dimensional right parasternal transthoracic echocardiographic examination of atrial septum and superior and inferior vena cavae. (A and B) Color Doppler examination in another patient showing four (numbered 1 through 4) separate
secundum defects at different levels of the atrial septum; (C and D) Two of these defects are viewed en face. In this 25-year-old female,
four atrial septal defects at different levels of the atrial septum could be demonstrated by sequential cropping of the three-dimensional
dataset. Only two of these defects could be visualized by two-dimensional transthoracic echocardiography. (LA: Left atrium; RA: Right
atrium). (Movie clip 74.1).
Source: Reproduced with permission from Patel V, Nanda NC, Upendram S, et al. Live three-dimensional right parasternal and supraclavicular transthoracic echocardiographic examination. Echocardiography. 2005;22:34960.
1736
Figs 74.2A and B: Live three-dimensional transthoracic echocardiographic (3D TTE) assessment of atrial septal defect. Arrowhead
points to a large secundum atrial septal defect visualized from both right (RA, A) and left atrial (LA, B) aspects. Note the large rim of
tissue surrounding the defect. (AS: Atrial septum). Movie clip 74.2, Part 1 shows en face viewing of a small atrial septal defect from both
the left and right atrial aspects. There is an ample rim of tissue surrounding the defect. Movie clip 74.2, Part 2 from another adult patient
with a secundum atrial septal defect. Regular and QLab (Philips Medical Systems, Andover, MA) cropping of the 3D data set views the
large defect en face (two arrowheads). Reasonable amount of atrial septal tissue surrounds the defect. The defect measures 3.2 3.1
cm, area 9.2 cm2. Movie clip 74.2, Part 3 from another patient with a secundum atrial septal defect. The two-dimensional study shows a
large defect (arrowhead) in the apical 4-chamber and subcostal views. Regular and QLab croppings view the large defect en face (two
arrowheads/arrows). Although the defect is similar in size to the previous patient, there is hardly any rim of the tissue adjacent to the
aorta making it hazardous to close it in the catheterization laboratory. Movie clip 74.2, Parts 4 and 5. In this large patient, a secundum
defect in the atrial septum (AS) was suspected during subcostal examination but a definitive diagnosis could not be made. When the
images were acquired using a three-dimensional transducer and cropped, the defect (arrow) was clearly visualized with left and right
shunting. (LA: Left atrium; RA: Right atrium). [Movie clip 74.2, (Parts 1 to 5)].
Source: Reproduced with permission from Mehmood F, Vengala S, Nanda NC, et al. Usefulness of live three-dimensional transthoracic
echocardiography in the characterization of atrial septal defects in adults. Echocardiography J. 2004;21:70713.
1737
Figs 74.4A and B: Live three-dimensional transthoracic echocardiography assessment of patent foramen ovale (PFO). (A and B)
Arrow shows the PFO while the arrowheads point to contrast signals moving through the defect into the left atrium (LA) following an
intravenous agitated saline injection. 3D TTE, three-dimensional transthoracic echocardiographic; (LV: Left ventricle).
Source: Reproduced with permission from Mehmood F, Vengala S, Nanda NC et al. Usefulness of live three dimensional transthoracic
echocardiography in the characterization of atrial septal defects in adults. Echocardiography J. 2004;21:70713.
Figs 74.5A to D
1738
Fig. 74.6: Transesophageal echocardiogram in the supine position. Echogram taken in the supine position showing the foramen
ovale is closed (right figure), and no apparent right-to-left shunt by
Doppler color flow (left figure). (LA: Left atrium; RA: Right atrium).
Source: Reproduced with permission from Sasaki T, Miyasaka Y,
Suwa Y, et al. Real time three-dimensional transesophageal echocardiographic images of platypnea-orthodeoxia due to patent foramen ovale. Echocardiography. 2013;30:E11617.
Fig. 74.7: Transesophageal echocardiogram in the sitting position. Echogram taken in the sitting position showing the foramen
ovale is wide open (right figure), with a massive right-to-left shunt
across the patent foramen ovale by Doppler color flow (left figure).
(LA: Left atrium; RA: Right atrium).
Source: Reproduced with permission from Sasaki T, Miyasaka Y,
Suwa Y, et al. Real time three-dimensional transesophageal echocardiographic images of platypnea-orthodeoxia due to patent foramen ovale. Echocardiography. 2013;30:E11617.
1739
Figs 74.8A to F: Live three-dimensional transthoracic echocardiographic assessment of transcatheter closure of atrial septal defect.
(A) Arrow points to the waist of the atrial septal defect (ASD) transcatheter closure device; (B and C) Shows the device viewed from the
top (B) and obliquely (C). Note that the left atrial disc (# 1) is larger than the right atrial disc (# 2); (D) Arrowhead points to the stainless
steel screw thread located in the center of the right atrial disc viewed enface; (E) Arrow points to the small residual shunt seen one day
after the device was positioned; (F) Amplatzer device used for transcatheter closure of ASD. Arrowhead points to the metallic cap and
the arrow points to the waist of the device. (LA: Left atrium; LV: Left ventricle; MC: Metallic cap; NW: Nitinol winding; RA: Right atrium;
RV: Right ventricle; ST: Screw thread). Movie clip 74.8 shows 3D TTE examination of the atrial septal defect closure device 6 weeks
after implantation. The device is well seated. (Movie clip 74.8).
Source: Reproduced with permission from Sinha A, Nanda NC, Misra V, et al. Live three-dimensional transthoracic echocardiographic
assessment of transcatheter closure of atrial septal defect and patent foramen ovale. Echocardiography. 2004;21:74953.
disc, the waist size of the device, and the distance between
the LA disc and the aorta.24 3D TEE measurements of LA
and RA disc lengths have been nearly identical to the
manufacturers assigned size, validating its calculations.
3D TEE is capable of not only selecting proper patients for
the percutaneous procedure, but also identifying patients
at higher risk of complications prompting more regular
follow-up.
Device embolization is a serious complication of
percutaneous repair that can be minimized by accurate
measurements of the ASD and placement of an appropriate
sized occluder device. Wei et al. described an unfortunate
1740
1741
Figs 74.12A to D
1742
F
Figs 74.12A to G: Live/real time, three-dimensional transesophageal echocardiographic assessment of device embolization
during percutaneous atrial septal defect closure. (A) The arrowheads
point to multiple secundum atrial septal defects (ASD, swiss cheese
appearance) viewed en face from the left atrium (LA); (B) Color
Doppler assessment showing flow signals within the defects viewed en
face (left panel). QLAB examination (right panel) showing four defects
numbered 1, 2, 3, and 4; (C) QLAB examination demonstrating en
face view of one of the defects (1) using Color Doppler. In the upper
left panel the cropping plane is positioned exactly parallel to the defect
which resulted in en face viewing of the defect in the lower left panel.
Subsequently the area was measured by planimetry; (D) Demonstrates
the first ASD closure device (D1) in position (viewed from left atrium
and anatomically correct). Arrowhead shows a large residual defect
viewed en face. The arrow points to the device placement catheter;
(E) Shows the second ASD closure device (D2) in position, partially
overlapping D1 (viewed from left atrium and anatomically correct).
Arrowhead shows the presence of one of the two significant residual
defects; (F) Shows embolization of one of the closure devices (D) to
the LA; (G) Shows the device (D) in the proximal descending thoracic
aorta (DA) after percutaneous manipulation from the iliac artery. 1 and
2 denote the right and the left atrial sides of the device, which are
viewed en face in the left lower and the right upper panels. (MV: Mitral
valve; RA: Right atrium).
Source: Reproduced with permission from Wei J, Hsiung MC,
Tsai SK, et al. Atrial septal occluder device embolization to an
iliac artery: A case highlighting the utility of three-dimensional
transesophageal echocardiography during percutaneous closure.
Echocardiography. 2012;29:112831.
1743
Figs 74.13A to C: Multiplane transesophageal 3D reconstruction of sinus venosus ASD. (A) The arrowhead points to the large defect in
the superior portion of the atrial septum. The arrow shows the right superior PV entering the SVC-atrial junction at the site of the defect.
(B and C) Orthogonal views demonstrating the size of the defect (ASD), which measured 3.69 cm2 in area. The maximal dimension of
the defect was 2.15 cm, which corresponded to the diameter of 2 cm measured at surgery. The top arrowhead in B points to the right
superior PV, and the bottom arrowhead points to the defect. (ASD: Atrial septal defect; SVC: Superior vena cava; LA: Left atrium; RA:
Right atrium).
Source: Reproduced with permission from Nanda NC, Ansingkar K, Espinal M, et al. Transesophageal three-dimensional echo assessment of sinus venosus atrial septal defect. Echocardiography. 1999;16:8357.
1744
1745
Figs 74.15A and B: (A) The arrowhead points to a perimembraneous ventricular septal (VS) defect viewed en face; (B) Shows color
Doppler flow signals in the defect. (LA: Left atrium; LVOT: Left ventricular outflow tract; MV: Mitral valve; RV: Right ventricle). [Movie clip
74.15, (Parts 1 to 4)].
Source: Reproduced with permission from Mehmood F, Miller AP, Nanda NC, et al. Usefulness of live/three-dimensional transthoracic
echocardiography in the characterization of ventricular septal defects in adults. Echocardiography. 2006;23:4217.
Figs 74.16A and B: (A and B) Ventriuclar septal defect. The arrow in A points to a large defect in the trabecular ventricular system
visualized in the apical four-chamber view. The arrow in B shows the same defect viewed en face by cropping of the three-dimensional
data set. Note the generous margins of the defect. Color Doppler examination shows flow signals moving through the defect. (Movie
clip 74.16).
Source: Reproduced with permission from Nanda NC, Hsiung MC, Miller AP, et al. Live/real time 3D echocardiography. Chichester, West
Sussex: Wiley, Blackwell, 2010.
1746
1747
Figs 74.18A to G: Perimembranous ventricular septal defect (inlet) (arrow). (A) Real time 3D echocardiography (RT3DE) volume-rendered image of the right ventricle (RV) displaying the right aspect of the ventricular septal defect. The location of the defect in relation to
the tricuspid valve (TV) is shown; (B) Surgical view of VSD from the right aspect; (C) RT3DE volume-rendered image of the left ventricle
(LV) displaying the left aspect of the VSD. The location of the defect in relation to the left ventricular outflow tract (LVOT) is shown; (D)
2DE parasternal long-axis views showed the VSD in relation to aortic valve and right ventricle; (E to G) Live three-dimensional transthoracic echocardiography in a patient with tetralogy of Fallot; (E) Note the wide aortic root (AO) and narrow pulmonary artery (PA); (F) The
AO overrides the interventricular septum (IVS); (G) Four-chamber view. The ventricular septal defect (VSD) is located at the crux. (AO:
ZAorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium; RV: Right ventricle);
Source: Figures 18A to D reproduced with permission from Chen FL, Hsiung NC, Nanda NC, Hsieh KS, Chou MC. Real time 3-dimensional echocardiography in assessing ventricular septal defects: An echocardiographic-surgical correlative study. Echocardiography.
2006;23:5628.
Source: Figures 18E to G reproduced with permission from Wang et al. Echocardiography. 2003;20:593604.
COMMON ATRIUM
Common atrium is a type of ASVD that occurs as a
result of absent atrial septal tissue and affixing of the
1748
Figs 74.19A to F
Figs 74.20A to D
1749
1750
Figs 74.20A to E: Live/real time, three-dimensional transthoracic echocardiography in partial atrioventricular septal defects.
(A) Arrowhead points to a prominent cleft in the anterior leaflet
of the left atrioventricular valve (LAV); (B) Arrows point to two
left ventricular papillary muscles located close to each other; (C)
Arrowhead points to a widened anteroseptal commissure (cleft)
of the right atrioventricular valve (RAV); (D) Arrowhead points to an
accessory LAV orifice; (E) Arrows point to the presence of only two
scallops in the posterior leaflet of LAV. The black arrowhead points
to the anterior leaflet of LAV. (AS: Anterosuperior leaflet; IB: Inferior bridging leaflet; L: Liver; LA: Left atrium; LV: Left ventricle; MI:
Mural inferior leaflet; ML: Mural lateral leaflet; PA: Pulmonary
artery; PV: Pulmonary valve; RA: Right atrium; RV: Right ventricle;
RAV: Right atrioventricular valve; RVO: Right ventricular outflow
tract; SB: Superior bridging leaflet). Movie clip 74.20C.
Source: Reproduced with permission from Singh A, Romp RL,
Nanda NC, et al. Usefulness of live/real time three-dimensional
transthoracic echocardiography in the assessment of atrioventricular septal defects. Echocardiography. 2006;23:598606.
Figs 74.21A to D: Live/real time, three-dimensional transthoracic echocardiography (3DTTE) in atrioventricular septal defects (AVSDs).
(A) Complete AVSD. Arrowhead shows attachment of CAV to the crest of the ventricular septum (Rastelli type A). Arrow points to atrial
component of the defect; (B) Complete AVSD. Arrowhead points to an anomalous papillary muscle projecting into the left ventricular
outflow tract causing subaortic obstruction; (C and D) Intermediate AVSD; (C) En face view of CAV shows superior bridging (SB) leaflet crossing over into the RV; (D) Both the AO and the PA are seen arising from the RV consistent with double outlet right ventricle.
(AS: Anterosuperior leaflet; IB: Inferior bridging leaflet; L: Liver; LA: Left atrium; LV: Left ventricle; MI: Mural inferior leaflet; ML: Mural
lateral leaflet; PA: Pulmonary artery; PV: Pulmonary valve; RA: Right atrium; RV: Right ventricle; RAV: Right atrioventricular valve; RVO:
Right ventricular outflow tract; SB: Superior bridging leaflet). (Movie clips 74.21B and C).
Source: Reproduced with permission from Singh A, Romp RL, Nanda NC, et al. Usefulness of live/real time three-dimensional transthoracic echocardiography in the assessment of atrioventricular septal defects. Echocardiography 2006;23:598606.
AORTOPULMONARY WINDOW
An aortopulmonary window is a rare congenital anomaly
involving a communication between the ascending
aorta and the pulmonary artery. Echocardiography is the
imaging modality of choice in diagnosing aortopulmonary
window.39 The typical echocardiographic finding in
patients with an aortopulmonary window is dilation of the
Figs 74.22A to D
1751
1752
Figs 74.22A to I: Three-dimensional transesophageal echocardiographic examination in cor triatriatum with common atrium.
(A and B) The arrow points to the cor triatriatum membrane while
the arrowheads (black) outline a large nonobstructive opening
present within the membrane; (C) The common atrium (CA) with
no atrial septum and the cor triatriatum membrane are well seen;
(D to H) Show left and right upper pulmonary veins (LPV and RPV)
located superior to the cor triatriatum membrane (arrows). 1 and
2 represent jets of right and left atrioventricular valve (RAV and
LAV) regurgitation, respectively. Portions of both jets appear to
move into the common atrium through the large opening in the cor
triatriatum membrane. Short-axis view of left atrioventricular valve
showing a cleft (C) in the anterior leaflet. (AV: Aortic valve; LVO:
Left ventricular outflow; PL: Posterior left atrioventricular valve
leaflet. RV: Right ventricle; LV: Left ventricle).
Source: Reproduced with permission from Baweja G, Nanda NC,
Kirklin JK. Definitive diagnosis of cor triatriatum with common
atrium by three-dimensional transesophageal echocardiography
in an adult. Echocardiography. 2004;21:3036.
1753
Figs 74.23A to F: Live/real time, three-dimensional transthoracic echocardiographic assessment of aortopulmonary window.
(A) Aortic short-axis view showing no evidence of an aortopulmonary window at this level. Movie clip 74.23, Part 2. The arrow points to
the aortopulmonary window; (B) When the three-dimensional dataset was cropped posteroanteriorly, a large communication between
the aorta (AO) and pulmonary artery (PA) was revealed (arrowhead); (C to E) The aortopulmonary window (arrowhead) could be viewed
en face by cropping the 3D dataset from the side (C) and rotating it (D and E); (F) Color Doppler exam shows mild pulmonic regurgitation
(PR). The arrowhead points to the aortopulmonary window. (AV: Aortic valve; LA: Left atrium; LPA: Left pulmonary artery; RPA: Right
pulmonary artery). [Movie clip 74.23, (Parts 1 and 2)].
Source: Reproduced with permission from Singh A, Mehmood F, Romp RL, Nanda NC, Mallavarapu RK. Live/real time three-dimensional transthoracic echocardiographic assessment of aortopulmonary window. Echocardiography. 2008;25:969.
1754
Figs 74.24A to D: Live/real time, three-dimensional transthoracic echocardiographic assessment of aortopulmonary window. (A and B)
Arrowhead points to the aortopulmonary window. Movie clip 74.24, Parts 1 to 2. The arrow points to the aortopulmonary window. The
asterisk in Movie clip 74.24, Part 3 denotes the descending thoracic aorta; (C) Arrow points to the interrupted aortic arch. Arrowhead
points to the aortopulmonary window; (D) Arrow points to a patent foramen ovale. (BCT: Brachiocephalic trunk; LCC: Left common
carotid artery; LSA: Left subclavian artery; MV: Mitral valve; RA: Right atrium; TV: Tricuspid valve). [Movie clip 74.24, (Parts 1 to 3)].
Source: Reproduced with permission from Singh A, Mehmood F, Romp RL, Nanda NC, Mallavarapu RK. Live/real time three-dimensional transthoracic echocardiographic assessment of aortopulmonary window. Echocardiography. 2008;25:969.
CONOTRUNCAL ANOMALIES
Transposition of the Great Arteries
Transposition of the great arteries (TGA) is organized
into two distinct types: dextro (D-TGA) and levo (L-TGA;
1755
Figs 74.25A to C: Two-dimensional transthoracic echocardiography in an adult with patent ductus arteriosus. (A and B) Color
Doppler examination demonstrates flow signals (arrowhead)
moving between the main pulmonary artery (PA) and the descending
thoracic aorta (DA) indicative of patent ductus arteriosus; (C)
Color Doppler-guided continuous-wave Doppler examination
demonstrates flow signals moving from PA to DA in systole and
back into PA in diastole (arrowheads). The arrow points to the
continuous-wave Doppler cursor line. (AO: Aorta; DA: Descending
aorta; PA: Pulmonary artery; RPA: Right pulmonary artery).
Source: Reproduced with permission from Sinha A, Nanda
NC, Khanna D, et al. Live three-dimensional transthoracic
echocardiographic delineation of patent ductus arteriosus. Echocardiography. 2004;21:4438.
A
Figs 74.26A and B
1756
Figs 74.26A to C: Live three-dimensional transthoracic echocardiography in an adult with patent ductus arteriosus. B mode images.
(A) The pyramidal section has been cropped to show the full extent
of the PDA (arrowhead) which connects the PA to DA. The arrow
points to the left atrial appendage; (B and C) The pyramidal section
has been cropped from the top to show the opening of the PDA
(arrowhead) into the pulmonary artery and its close location to the
origin of the left pulmonary artery (arrow). (LPA: Left pulmonary
artery; LV: Left ventricle; PV: Pulmonary valve).
Source: Reproduced with permission from Sinha A, Nanda NC,
Khanna D, et al. Live three-dimensional transthoracic echocardiographic delineation of patent ductus arteriosus. Echocardiography. 2004;21:4438.
Figs 74.27A to D
1757
F
Figs 74.27A to G: Live three-dimensional transthoracic echocardiography in an adult with patent ductus arteriosus (PDA). Color Doppler
images. The pyramidal section has been cropped to visualize flow
signals in PA, PDA (arrowhead), and DA. (A) Shows the length of the
PDA (arrowhead) connecting the PA and DA. The arrow points to an
intercostal artery arising from DA; (B and C) Color Doppler images have
been isolated by completely suppressing B mode images. The isolated
color Doppler images could be rotated from 0 to 176, thus enabling
comprehensive visualization of flows in PA, PDA (arrowhead), and DA in
three-dimensions. Images at 0 (top left), 45 (top right), 90 (lower left),
and 176 (lower right) rotation are shown; (D) Frontal sections showing
color Doppler signals moving from PA into DA in systole (left) and back
into PA in diastole (right); (E and F) Tilted (top half) and enface (lower half)
views of PDA (arrowheads) at aortic (E) and pulmonary (F) connections.
On the right, B mode signals have been suppressed resulting in only
flow visualization; (G) Schematic L, length of the ampulla, defined as the
distance between the mid-portion of narrowest diameter of PDA and the
mid-portion of the aortic end. This measured 0.94 cm in our patient. D,
PDA diameter at aortic insertion (ampulla diameter). This measured 1.31
cm in our patient. (DA: Descending thoracic aorta; LPA: Left pulmonary
artery; LV: Left ventricle; PV: Pulmonary valve; RV: Right ventricle).
(Movie clip 74.27).
Source: Reproduced with permission from Sinha A, Nanda NC, Khanna
D, et al. Live three-dimensional transthoracic echocardiographic
delineation of patent ductus arteriosus. Echocardiography. 2004;21:
4438.
Figs 74.29 to 74.37). D-TGA is characterized by ventriculoarterial discordance whereby the right ventricle
pumps blood to the aorta and the left ventricle pumps
blood to the pulmonary artery.43 A shunt lesion (ASD,
VSD, or PDA) between the left and right side of the
heart must be present to ensure appropriate mixing of
pulmonary and systemic return to enable perfusion of
oxygenated blood to the bodys vital organs. In an atrial
switch surgery, deoxygenated blood is diverted from the
inferior and superior vena cavae to the left side of the heart
and from there into the pulmonary artery through the
creation of an intra-atrial baffle.44 Senning and Mustard
atrial switch procedures were historically the standard of
1758
Fig. 74.28: Thoracic magnetic resonance angiogram with Gadolinium in the same patient as Figures 25 to 27. The arrowhead
points to patent ductus arteriosus (PDA) viewed in the left lateral
position. (DA: Descending thoracic aorta; PA: Pulmonary artery;
RV: Right ventricle).
Source: Reproduced with permission from Sinha A, Nanda
NC, Khanna D, et al. Live three-dimensional transthoracic
echocardiographic delineation of patent ductus arteriosus.
Echocardiography. 2004;21:4438.
Figs 74.29A and B: Dextro-transposition of the great arteries. (A) The aorta (AO) arises from the right ventricle (RV); (B) The pulmonary
artery (PA) originates from the left ventricle (LV). The arrows point to left and right branches of the main PA. (Movie clips 74.29A and B).
Source: Reproduced with permission from Enar S, Singh P, Douglas C, et al. Live/real time three-dimensional transthoracic echocardiographic assessment of transposition of the great arteries in the adult. Echocardiography. 2009;26:1095104.
1759
Figs 74.30A and B: Levo-transposition (corrected transposition) of the great arteries. (A) The arrows point to two vena contractas of
tricuspid regurgitation (TR) jets; (B) En face view of the two TR vena contractas (arrows). The movie clips show cropping of the tricuspid
regurgitation jet (arrow) to view the vena contracta (arrowhead) en face in another patient with levo-transposition (corrected transposition) of the great vessels. (RA: Right atrium; RV: Right ventricle; TV: Tricuspid valve). [Movie clip 74.30, (Parts 1 to 3)].
Source: Reproduced with permission from Enar S, Singh P, Douglas C, et al. Live/real time three-dimensional transthoracic echocardiographic assessment of transposition of the great arteries in the adult. Echocardiography. 2009;26:1095104.
1760
1761
Figs 74.34A to D: Dextro-transposition of the great arteries. (A and B) The intra-atrial baffle (B) appears as a shelf when viewed en
face by cropping from the bottom. A defect in the baffle is not well seen in the illustration but it is clearly visualized (arrowhead) in the
accompanying movie clips which view the baffle en face; (C) Shows the relationship of the baffle to the inferior vena cava (IVC); (D)
Color Doppler study shows systemic venous flow signals (arrow) moving through the left ventricle (LV) toward the pulmonary artery (PA).
(SVA: Systemic venous atrium). [Movie clip 74.34, (Parts 1 and 2)].
Source: Reproduced with permission from Enar S, Singh P, Douglas C, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of transposition of the great arteries in the adult. Echocardiography. 2009;26:1095104.
1762
1763
Tetralogy of Fallot
ToF is a complex cyanotic CHD defect that includes VSD,
overriding aorta, pulmonary outflow tract obstruction and
right ventricular hypertrophy. Surgical repair is necessary
early in life and these patients require close followup to monitor for possible complications. Postsurgical
ToF patients can experience severe pulmonary valve
regurgitation or right ventricular outflow obstruction.
Very rarely, patients may have residual VSDs.48 3D TTE
has demonstrated its superiority compared to 2D imaging
in assessing pulmonary regurgitation severity based on
visualization of the vena contracta.49 Accurate assessment
of the size and shape of residual defects is crucial for
determining if repeat surgery is necessary and 2D TTE,
especially in the setting of other congenital anomalies, has
1764
D
Figs 74.38A to E: Two-dimensional transesophageal and transthoracic echocardiography in an adult with anomalous origin of the left
coronary artery from the pulmonary artery. (A and B) Intraoperative
transesophageal study shows a dilated right coronary artery (RCA, A)
and the surgically created tunnel (T, B); (C to E) Transthoracic study
(performed 13 years after surgery). The arrow in C shows a small
defect in the tunnel (T) near the aorta through which shunting occurs
into the pulmonary artery (arrowhead in D). Color Doppler-guided
continuous-wave Doppler examination (E) shows shunting occurring
practically throughout the cardiac cycle (arrowheads). The arrow in
E points to the Doppler cursor line. (AO: Aorta; LA: Left atrium; LPA:
Left pulmonary artery; PR: Pulmonary regurgitation; PV: Pulmonary
valve; RA: Right atrium; RPA: Right pulmonary artery; RVOT: Right
ventricular outflow tract). Source: Reproduced with permission from
Ilgenli TF, Nanda NC, Sinha A, Khanna D. Live three-dimensional
transthoracic echocardiographic demonstration of anomalous origin
of left coronary artery from the pulmonary artery. Echocardiography.
2004;21:55962.
Figs 74.39A to F
1765
1766
Figs 74.39A to G: Live three-dimensional transthoracic echocardiographic demonstration of anomalous origin of left coronary
artery from the pulmonary artery. (A) The arrowhead points to the
orifice of the anomalous coronary artery while the arrow shows
the defect in the tunnel; (B and C) Color Doppler examination.
In B, flow signals (arrowhead) are seen moving into the orifice
of the anomalous coronary artery from the tunnel (T). The arrow
points to the tunnel-pulmonary artery shunt. In C, the arrow points
to color Doppler shunt flow signals visualized in three dimensions;
(D) Coronary angiogram. Arrowhead points to fistula originating
from the left anterior descending coronary artery (LAD) and
draining into the pulmonary artery (PA) which is partially opacified.
(E to G) Live three-dimensional transthoracic echocardiography; E.
Arrowhead points to a small localized area of abnormal flow signals
in the pulmonary artery (PA) just distal to the pulmonary valve (PV)
in both diastole (F) and systole (G) consistent with entrance of
the fistula into the PA. The arrow in F points to mild pulmonary
regurgitation. (AV: aortic valve; CX: Circumflex coronary artery;
LM: Left main coronary artery; RVO: Right ventricular outflow
tract). (PA: Main pulmonary artery; AO: Aorta; LA: Left atrium;
LPA: Left pulmonary artery; PR: Pulmonary regurgitation; PV:
Pulmonary valve; RA: Right atrium; RPA: Right pulmonary artery;
RVOT: Right ventricular outflow tract). Source: Figure 74.39A to C
Reproduced with permission from Ilgenli TF, Nanda NC, Sinha A,
Khanna D. Live three-dimensional transthoracic echocardiographic
demonstration of anomalous origin of left coronary artery from the
pulmonary artery. Echocardiography. 2004;21:559562. Source:
Figure 74.39D Reproduced with permission from Mehta D, Nanda
NC, Vengala S, Mehmood F, Taylor J. Live three dimensional
transthoracic echocardiographic demonstration of coronary artery
to pulmonary artery fistula. Am J Geriatric Cardiol. 2005;14:424.
Source: Figures 74.39E to G Reproduced with permission from
Mehta D, Nanda NC, Vengala S, Mehmood F, Taylor J. Live three
dimensional transthoracic echocardiographic demonstration of
coronary artery to pulmonary artery fistula. Am J Geriatric Cardiol.
2005;14:424.
1767
Figs 74.40A and B: Live/real time, three-dimensional transthoracic echocardiographic detection of left main coronary artery fistula into
the left ventricle. (A and B) The arrowhead in A points to the enlarged left main coronary artery (LM). Its entrance into the left ventricle
(LV) is denoted by an arrowhead in (B). (AO: Aorta). (Movie clips 74.40A and B).
Figs 74.41A and B: Two-dimensional transthoracic echocardiography in an adult patient with bicuspid aortic valve and severe aortic
regurgitation. (A) The bicuspid aortic valve (AV) is shown in systole in the open position with no evidence of stenosis; (B) Color Doppler
examination shows an eccentric jet of aortic regurgitation originating posteriorly (horizontal arrow). The vertical arrow points to mild
pulmonic regurgitation. (PV: Pulmonic valve; RA: Right atrium; RV: Right ventricle). (Movie clips 41A and B).
Source: Reproduced with permission from Singh P, Dutta R, Nanda NC. Live/real time three-dimensional transthoracic echocardiographic assessment of bicuspid aortic valve morphology. Echocardiography. 26.:4:47880.
1768
Figs 74.42A and B: Live/real time, three-dimensional transthoracic echocardiography in the same patient. (A) Note the presence of
several folds in the bicuspid aortic valve in the closed position viewed from the ventricular side; (B) The arrow points to a well circumscribed perforation in the posterior cusp of the aortic valve. (LA: Left atrium; PV: Pulmonic valve; RA: Right atrium; RV: Right ventricle).
(Movie clips 74.42A and B).
Source: Reproduced with permission from Singh P, Dutta R, Nanda NC. Live/real time three-dimensional transthoracic echocardiographic assessment of bicuspid aortic valve morphology. Echocardiography. 26;4:47880.
Figs 74.43A and B: Two-dimensional transthoracic echocardiography. (A and B) The aortic valve (AV) appears bicuspid. (LA: Left
atrium; RV: Right ventricle). (Movie clip 74.43).
Source: Reproduced with permission from Burri MV, Nanda NC, Singh A, Panwar SR. Live/real time three-dimensional transthoracic
echocardiographic identification of quadricuspid aortic valve. Echocardiography. 2007;24:6535.
1769
Figs 74.45A and B: Live/real time, three-dimensional transthoracic echocardiography. (A and B) Shows a quadricuspid aortic valve
with four numbered leaflets clearly visualized. (AO: Aorta, RA: Right atrium; TV: Tricuspid valve.). [Movie clip 74.45, (Parts 1 and 2)].
Source: Reproduced with permission from Burri MV, Nanda NC, Singh A, Panwar SR. Live/real time three-dimensional transthoracic
echocardiographic identification of quadricuspid aortic valve. Echocardiography. 2007;24:6535.
1770
aortic valve. This is unlike a 3DE data set that includes the
whole valve which can then be studied at any time using
any desired plane and angulation.
Subaortic Stenosis
Stenosis can occur not only at the level of the valve but
above the valve (supravalvular) as well as below the valve
(subvalvular or subaortic). When subaortic stenosis is
diagnosed it is important to distinguish the fixed from
the dynamic type. Fixed subaortic stenosis can be due to
a focal, fibromuscular membrane or a diffuse tunnel-type
lesion. On the other hand, dynamic subaortic stenosis can
develop as a result of systolic anterior motion of the mitral
valve, a common echocardiographic finding in patients
with hypertrophic cardiomyopathy.1 Interestingly, there is
a strong association between patients previously diagnosed
with a VSD and subaortic stenosis which appears to be
related to fibrous tissue formation from turbulent blood
flow at the prior surgical site.58
With 3D TTE, the aortic valve, subaortic membrane,
and surrounding cardiac anatomy is clearly visualized
which is beneficial in measuring the severity of stenosis
(Figs 74.46 and 74.47).1,59,60 2D TTE can be helpful
in diagnosing subaortic stenosis based on Doppler
measurement of gradient across the membrane, however,
these calculations are often inaccurate. In a study by
Figs 74.46A and B: Two-dimensional transthoracic echocardiogram in discrete subaortic membranous stenosis. (A) The arrowhead
points to the subaortic membrane; (B) Using color Doppler-guided continuous-wave Doppler, a peak gradient (PG) of 134 mm Hg
(arrow) was obtained across the left ventricular (LV) outflow tract. (AO: Aorta; LA: Left atrium; RV: Right ventricle; RVO: Right ventricular
outflow tract).
Source: Reproduced with permission from Agrawal GG, Nanda NC, Htay T, Dod HS. Live three-dimensional transthoracic echocardiographic identification of discrete subaortic membranous stenosis. Echocardiography. 2003;20:6179.
1771
Figs 74.47A to C: Live three-dimensional transthoracic echocardiography in discrete subaortic membranous stenosis.
(A and B) The arrowhead points to a narrow opening in the
membrane imaged in short axis; (C) Membrane (arrowhead)
viewed from the top showing its attachment to the ventricular
septum (VS) and the anterior leaflet of the mitral valve. (AO: Aorta;
RA: Right atrium). The arrowhead in the Movie clip 74.47 points to
the obstructing membrane. (Movie clip 74.47).
Source: Reproduced with permission from Agrawal GG, Nanda
NC, Htay T, Dod HS. Live three-dimensional transthoracic echocardiographic identification of discrete subaortic membranous
stenosis. Echocardiography. 2003;20:6179.
1772
Figs 74.49A and B: Discrete subaortic membrane. Two-dimensional transthoracic echocardiography. (A) Arrowhead points to the
membrane imaged in the parasternal long-axis view; (B) Shows peak and mean pressure gradients of 64 mm Hg and 31 mm Hg across
the left ventricular outflow tract by continuous-wave Doppler. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle). (Movie
clip 74.49).
Source: Reproduced with permission from Bandarupalli N, Faulkner M, Nanda NC, Pothineni KR. Erroneous diagnosis of significant
obstruction by Doppler in a patient with discrete subaortic membrane: Correct diagnosis by 3D-transthoracic echocardiography. Echocardiography. 2008;25:10046.
Figs 74.50A and B: Discrete subaortic membrane. Live/real time, three-dimensional transthoracic echocardiography. (A and B) Subaortic membrane (arrowhead) and orifice viewed en face. The orifice measured 2.29 cm2 by planimetry. (LA: Left atrium; PV: Pulmonic
valve; RA: Right atrium; RV: Right ventricle). (Movie clip 74.50).
Source: Reproduced with permission from Bandarupalli N, Faulkner M, Nanda NC, Pothineni KR. Erroneous diagnosis of significant
obstruction by Doppler in a patient with discrete subaortic membrane: Correct diagnosis by 3D-transthoracic echocardiography. Echocardiography. 2008;25:10046.
1773
Fig. 74.51: Discrete subaortic membrane. Live/real time, threedimensional transthoracic echocardiography. Aortic valve orifice
viewed en face. It measured 2.94 cm2 by planimetry. (AO: Aorta;
LA: Left atrium; LV: Left ventricle; RV: Right ventricle).
Source: Reproduced with permission from Bandarupalli N,
Faulkner M, Nanda NC, Pothineni KR. Erroneous diagnosis of
significant obstruction by Doppler in a patient with discrete
subaortic membrane: Correct diagnosis by 3D-transthoracic
echocardiography. Echocardiography. 2008;25:10046.
Fig. 74.52: Real time, two-dimensional transesophageal echocardiogram in aortic arch vasum vasi to pulmonary artery fistula. The
arrowhead points to flow signals moving from the wall of the aortic
arch (AO) into the main PA. (Movie clip 74.52).
Source: Reproduced with permission from Sadat K, Pradhan
M, Nanda NC, Joshi D, Diddi HP. Two- and three-dimensional
transthoracic echocardiography in the assessment of aortic
arch vasum vasi to pulmonary artery fistula. Echocardiography.
2013;30:21924.
1774
Figs 74.53A and B: Live/real time, three-dimensional transthoracic echocardiogram in aortic arch vasum vasi to pulmonary artery
fistula. (A) The arrowhead points to abnormal flow signals originating within the posterior wall of the aortic arch. A small area of flow
acceleration is also noted. Careful cropping revealed no connection of this abnormal flow signals with the aortic lumen; (B) En face view of
the fistula vena contracta. It is very small measuring 1.7 mm in diameter, area 3.5 mm2. (LA: Left atrium). (Movie clips 74.53A and B).
Figs 74.54A and B: Live/real time, three-dimensional transthoracic echocardiography in cor triatriatum sinister. (A) Arrowhead points
to cor triatriatum membrane (M), which is located superior to left atrial appendage (LAA); (B) Arrowhead (arrow in movie) points to a
large opening in cor triatriatum membrane visualized en face. The dimensions were 3.06 1.03 cm and area 2.3 cm2. (LA: Left atrium;
LV: Left ventricle; LVO: Left ventricular outflow tract; RA: Right atrium; RV: Right ventricle). (Movie clip 74.54).
Source: Reproduced with permission from Patel V, Nanda NC, Arellano I, Yelamanchili P, Rajdev S, Baysan O. Cor triatriatum sinister:
Assessment by live/real time three-dimensional transthoracic echocardiography. Echocardiography. 2006;23:8012.
1775
B
Figs 74.55A to C: Live three-dimensional transthoracic echocardiographic assessment of isolated cleft mitral valve. The
arrowhead points to the cleft in the anterior mitral valve leaflet
seen in the open (A and B) and closed (C) positions. (LV: Left
ventricle; PML: Posterior mitral leaflet; RV: Right ventricle). The
cleft is directed toward the left ventricular outflow tract unlike
the atrioventricular septal defect where the cleft points medially.
Movie clip 74.55, Parts 1 and 2 from another patient shows an
isolated cleft (arrow) in the anterior mitral leaflet in a 28-year-old
female. Color Doppler examination shows prominent regurgitation
(arrowhead) into the left atrium through the cleft. [Movie clip 74.55,
(Parts 1 and 2)].
Source: Reproduced with permission from Sinha A, Kasliwal
RR, Nanda NC, et al. Live three-dimensional transthoracic
echocardiographic assessment of isolated cleft mitral valve.
Echocardiography. 2004;21:65761.
Ebsteins Anomaly
1776
Figs 74.56A to D: Live three-dimensional transthoracic echocardiographic assessment of isolated cleft mitral valve. (A to C) The
arrowhead points to the cleft in the anterior mitral valve leaflet. Note thickened mitral leaflets with a narrow orifice indicative of associated
mitral stenosis; (D) Isolated cleft mitral valve in another child. This en-face transthoracic three-dimensional view shows the cleft (arrow)
dividing the superior and inferior components of the anterior mitral leaflet. Note the perception of the depth of the cleft with threedimensional imaging. (MV: Mitral valve; LV: Left ventricle; PML: Posterior mitral leaflet; RV: Right ventricle). (Movie clip 74.56D).
Source: Figures 74.56A to C reproduced with permission from Sinha A, Kasliwal RR, Nanda NC, et al. Live three-dimensional
transthoracic echocardiographic assessment of isolated cleft mitral valve. Echocardiography. 2004;21:65761.
OTHER ABNORMALITIES
Chiari Network
Chiari network is often clinically insignificant, but some
reports suggest increased risk of thrombus formation,
paradoxical embolization, or endocarditis.71 Chiari
network is composed of a large fenestrated membrane
in the right atrium with wide attachments at the crista
terminalis and interatrial septum as a result of persistence
of the right-sided sinus venosus valve. 3D TTE is valuable
in distinguishing Chiari network from Eustachian and
Thebesian valves based on their smaller size and location,
A
Figs 74.58A and B
1777
B
Figs 74.57A to C: Live/real time, three-dimensional transthoracic
echocardiography in Ebsteins anomaly associated with transposition of the great vessels. (A) Four-chamber view shows apparent
displacement of the attachment of the septal leaflet of the tricuspid
valve (TV) toward the apex; (B) Tethering of the septal leaflet of the
TV results in a bubble-like appearance (yellow arrowhead) in the
middle portion of the ventricular septum as the nontethered portion
moves toward closure during systole. This transverse section
was taken at a level denoted by #1 in (A); (C) Transverse section
taken at a more inferior level (#2 in A) demonstrates bubble-like
appearance of both septal (yellow arrowhead) and posterior (black
arrowheads) TV leaflets produced by tethering. (a: Anterior TV
leaflet; LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RA:
Right atrium; RV: Right ventricle).
Source: Reproduced with permission from Patel V, Nanda NC,
Rajdev S, et al. Live/real time three-dimensional transthoracic
echocardiographic assessment of Ebsteins Anomaly. Echocardiography. 2005;22:84754.
1778
Figs 74.58C to H
I
and cor triatriatum dexter network based on its thickness
with few or absent fenestrations (Figs 74.60 and 74.61).
Other clinical clues suggesting the diagnosis of cor
triatriatum dexter are obstruction of blood flow and its
frequent association with other congenital defects. 2D TTE
has diagnosed a defect as a Eustachian valve but followup imaging with 3D TTE confirmed the diagnosis of Chiari
network.71
1779
1780
1781
Fig. 74.60: Real time, two-dimensional transesophageal echocardiography. Arrowhead points to a linear structure in the right
atrium (RA) consistent with a Eustachian valve. Movie clip 74.60,
Part 2, arrow shows the tumor. [Movie clip 74.60, (Parts 1 and 2)].
Source: Reproduced with permission from Pothineni KR, Nanda
NC, Burri MV, Singh A, Panwar SR, Gandhari S. Live/real time
three-dimensional transthoracic echocardiographic visualization of
Chiari Network. Echocardiography. 2007;24:9957.
1782
A
Figs 74.63A and B
1783
Figs 74.63A to F: Live/real time, three-dimensional transesophageal echocardiography of right coronary artery to right atrium fistula.
(A) Qlab study. Top arrows and the bottom right arrow point to the origin of the dilated RCA. The RCA opening viewed en face (arrow
in bottom left panel) measured 1.70 cm 1.55 cm, area 1.96 cm2; (B) Vertical arrowhead shows the continuity of the descending and
the ascending (transverse arrowhead) limbs of the fistula. Arrow shows origin of the dilated RCA; (C) Qlab cropping also shows the
continuity (lower arrowhead) of the descending and ascending (upper right arrowhead) limbs of the fistula. Arrow in upper left and lower
right panels show the origin of dilated RCA; (D) The arrow shows flow signals moving from the fistula (arrowheads) into RA; (E and F)
Represent regular (E) and Qlab (F) en face views (arrow) of the opening of fistula into RA. This measured 1.83 cm 1.43 cm, area 3.94
cm2. Arrowheads show segments of the fistula. (AO: Aorta; AV: Aortic valve; RA: Right atrium). (Movie clips 74.63B to 74.63F).
Source: Reproduced with permission from Mishra J, Puri HP, Hsiung MC, et al. Incremental value of live/real time three-dimensional
over two-dimensional transesophageal echocardiography in the evaluation of right coronary artery fistula. Echocardiography. 2011;28:
8058.
1784
CONCLUSION
1785
Figs 74.64A to D: Live/real time, three- dimensional transesophageal echocardiography in ruptured right sinus of Valsalva aneurysm.
(A and B) Arrow points to site of rupture of the aneurysm (AN); (C and D) Arrow points to the mouth of the aneurysm viewed en face.
(AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle). (Movie clips 74.64A to 74.64D).
Source: Reproduced with permission from Raslan S, Nanda NC, Lloyd L, Khairnar P, Reilly SD, Homan WL. Incremental value of live/
real time three-dimensional transesophageal echocardiography over the two-dimensional technique in the assessment of sinus of
Valsalva aneurysm rupture. Echocardiography. 2011;28:10415.
1786
Figs 74.66A and B: Transesophageal echocardiographic delineation of ventricular septal aneurysm producing right ventricular outflow
obstruction in an adult. (A) The arrow points to the ventricular septal aneurysm bulging into right ventricular outflow tract. The ventricular
septal defect is delineated by the arrowhead; (B) Color Doppler examination shows a narrow turbulent jet (black arrowheads) indicative
of significant obstruction produced by the aneurysm (arrow). (AV: Aortic valve; LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery;
RA: Right atrium; TV: Tricuspid valve).
Source: Reproduced with permission from Baweja G, Nanda NC, Nekkanti R, Dod H, Ravi B, Fadel A. Three-dimensional transesophageal echocardiographic delineation of ventricular septal aneurysm producing right ventricular outflow obstruction in an adult. Echocardiography. 2004;21:957.
A
Figs 74.67A and B
1787
Figs 74.67A to D: Three-dimensional transesophageal echocardiographic delineation of ventricular septal aneurysm producing
ventricular outflow obstruction in the same patient as in previous figure. (A) Short-axis (en-face) view just below the level of the
pulmonary valve (PV) showing the aneurysm (arrow) practically occupying the entire right ventricular outflow tract (RVO) indicative of
severe obstruction; (B) Long-axis view also shows the aneurysm (arrow) bulging into the RVO; (C and D) Short-axis view of the PV
(black arrow); (C) Normal opening of the PV during systole indicative of absence of any obstruction at this level; (D) The PV in closed
position in diastole. VS, ventricular septum. (AV: Aortic valve; LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery; RA: Right atrium;
TV: tricuspid valve).
ACKNOWLEDGMENT
We thank Dr Maximiliano German Amado Escauela for
his help.
REFERENCES
1. Hage FG, Raslan S, Dean P, et al. Real time threedimensional transthoracic echocardiography in congenital
heart disease. Echocardiography. 2012; 29(2):22031.
2. Aqel RA, Hage FG, Cogar B, et al. Transthoracic echocardiography guided procedures in the catheterization
laboratory. Echocardiography. 2007;24(9):10007.
3. Shirali GS. Three-dimensional echocardiography in congenital heart disease. Echocardiography. 2012;29(2): 2428.
4. De Castro S, Caselli S, Papetti F, et al. Feasibility and
clinical impact of live three-dimensional echocardiography
in the management of congenital heart disease. Echocardiography. 2006;23(7):55361.
5. Salustri A, Spitaels S, McGhie J, et al. Transthoracic threedimensional echocardiography in adult patients with congenital heart disease. J Am Coll Cardiol. 1995;26(3):75967.
6. Bharucha T, Roman KS, Anderson RH, et al. Impact of
multiplanar review of three-dimensional echocardiographic
data on management of congenital heart disease. Ann
Thorac Surg. 2008;86(3):87581.
1788
34. Singh P, Mehta A, Nanda NC. Live/real time threedimensional transthoracic echocardiographic findings
in an adult with complete atrioventricular septal defect.
Echocardiography. 2010;27(1):8790.
35. Van den Boscha AE, Van Dijka VF, McGhiea JS, et al. Real
time transthoracic three-dimensional echocardiography
provides additional information of left-sided AV valve
morphology after AVSD repair International. J Cardiol.
2006;106:3604.
36. Kaza E, Marx GR, Kaza AK, et al. Changes in left
atrioventricular valve geometry after surgical repair of
complete atrioventricular canal. J Thorac Cardiovasc Surg.
2012;143(5):11171124.
37. Miller AP, Nanda NC, Aaluri S, et al. Three-dimensional
transesophageal echocardiographic demonstration of
anatomical defects in AV septal defect patients presenting
for reoperation. Echocardiography. 2003;20(1):1059.
38. Baweja G, Nanda NC, Kirklin JK. Definitive diagnosis
of cor triatriatum with common atrium by threedimensional transesophageal echocardiography in an
adult. Echocardiography. 2004;21(3):3036.
39. Apitz C, Kaulitz R, Sieverding L, et al. [Echocardiographic
diagnosis of the aorto-pulmonary window]. Ultraschall
Med. 2007;28(2):18994.
40. Singh A, Mehmood F, Romp RL, et al. Live/Real time threedimensional transthoracic echocardiographic assessment
of aortopulmonary window. Echocardiography. 2008;25
(1):969.
41. Campbell M. Natural history of persistent ductus arteriosus.
Br Heart J. 1968;30(1):413.
42. Sinha A, Nanda NC, Khanna D, et al. Live three-dimensional
transthoracic echocardiographic delineation of patent
ductus arteriosus. Echocardiography. 2004;21(5):4438.
43. Warnes CA. Transposition of the great arteries. Circulation.
2006;114(24):2699709.
44. Enar S, Singh P, Douglas C, et al. Live/real time threedimensional transthoracic echocardiographic assessment
of transposition of the great arteries in the adult. Echocardiography. 2009;26(9):1095104.
45. Skinner J, Hornung T, Rumball E. Transposition of the great
arteries: from fetus to adult. Heart. 2008;94(9):122735.
46. Nanda NC, Hsiung MC, Miller AP, et al. Live/Real Time
3D Echocardiography. Chichester, West Sussex: WileyBlackwell, 2010.
47. Ahmed S, Nekkanti R, Nanda NC, et al. Three-dimensional
transesophageal echocardiographic demonstration of
intraatrial baffle obstruction. Echocardiography. 2003;20
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48. Ho KW, Tan RS, Wong KY, et al. Late complications
following tetralogy of Fallot repair: the need for long-term
follow-up. Ann Acad Med Singap. 2007;36(11):94753.
49. Pothineni KR, Wells BJ, Hsiung MC, et al. Live/real time
three-dimensional transthoracic echocardiographic assessment of pulmonary regurgitation. Echocardiography. 2008;
25(8):9117.
50. Patel S. Normal and anomalous anatomy of the coronary
arteries. Semin Roentgenol. 2008;43(2):100112.
1789
1790
CHAPTER 75
Echocardiography in the Evaluation of
Adults with Congenital Heart Disease
Reema Chugh
Snapshot
Valvular Disease
Most of the fundamental ideas of science are essentially simple, and may,
as a rule, be expressed in a language comprehensible to everyone.
Albert Einstein
INTRODUCTION
Advancements in pediatric, medical care, surgical, and
interventional techniques have improved survival into
adulthood for over 85% of those born with congenital
heart defects (CHDs) in developed countries. Depending
upon the type of defect, presenting symptoms, and the
availability of medical resources, many are diagnosed in
infancy and childhood, while in others, the diagnosis is not
made until adulthood. Transthoracic echocardiography is
the primary diagnostic imaging test, while other modalities
such as cardiac catheterization, magnetic resonance
imaging (MRI), or computed tomographic angiography
(CTA) are used as confirmatory or complementary tests
for complete assessment and evaluation of extracardiac
structures in adults with congenital heart disease (ACHD).
While coronary angiography remains the gold standard for
assessment of coronary artery disease, MRI is the reference
standard for quantification of the right ventricular volume
1792
timely
(ACC)/
for the
disease
Table 75.2: Indications for Performing Transesophageal Echocardiography (TEE) for Adults with Congenital Heart Diseases
(ACHD)
Diagnostic indications
Identification of defects in adults with poor acoustic transthoracic windows
Identification of defects that have limited visibility on TTE
(such as a sinus venosus defect, fenestrated ASD, origin of
the coronaries)
Rule out cardioembolic source in an adult with stroke,
including assessment of possible right-to-left shunt due to
a PFO with an agitated saline contrast study; examination
of the left atrial appendage for a thrombus, atherosclerotic
plaques in the proximal aorta
Examination of intra- or extracardiac baffles following the
Fontan, Senning, or Mustard procedure
Evaluation of vegetation, perforation, endarteritis, or suspected abscess
Ruling out an intracardiac thrombus prior to cardioversion
for atrial flutter/fibrillation
Status of prosthetic valves
Guiding catheter-based interventions
Directing placement of ASD or VSD occlusion devices
accurate assessment of size, shape, location of the defect,
and identifying associated defects
Delineation of structures not clearly visible on transthoracic
echocardiography (such as pulmonary veins)
Catheter tip placement for dilation and stent implantantion
for conduit/baffle stenoses in catheterization laboratory
Perioperative indications
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
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Atrioventricular (AV) concordanceright atrium empties into the right ventricle, left atrium empties into the left ventricle
Atrioventricular (AV) discordanceright atrium empties into the left ventricle, left atrium empties into the right ventricle
Dextrocardiaapex of the heart points to the right (old term dextroversion)failure of pivoting of the cardiac apex to the left
Levocardiaapex of the heart points to the left
Mesocardiaapex of the heart points to the middle
Dextropositiondisplacement of the heart in the right chest due to a space occupying structure
Eisenmenger physiologydevelopment of irreversible pulmonary hypertension with equalization of pulmonary and systemic
pressures, with pulmonary vascular resistance exceeding systemic vascular resistance leading to flow reversal (right to left) through
an intracardiac shunt
Right isomerismbilateral right handedness (two morphological right atria), asplenia, both the aorta (anterior) and inferior vena
cava (posterior) are on the same side of the spine
Left isomerismbilateral left handedness (two morphological left atria), polysplenia, both the aorta (posterior) and inferior vena
cava (anterior) are on the same side of the spine
Situs inversus totalisheart is located in the mirror-image position of normal
Ventriculoarterial concordancethe morphological right ventricle pumps into the pulmonary artery, while the morphological left
ventricle pumps into the aorta
Ventriculoarterial discordancethe morphological right ventricle pumps into the aorta, while the morphological left ventricle
pumps into the pulmonary artery (transposition of the great arteries)
Restrictive VSDusually small in size with a left-to-right shunt without causing significant hemodynamic derangement
Nonrestrictive VSDlarge VSD at risk for reversal of the shunt (right-to-left). Hemodynamically, the pulmonary arterial and aortic
pressures are equal so the magnitude and direction of the shunt is determined by the pulmonary vascular resistance (PVR).
1794
Table 75.4: Common Surgical Terms in Adult Congenital Heart Disease (ACHD)
Classic BlalockTaussigThomas shuntsubclavian to pulmonary artery anastomosis to increase pulmonary blood flow
Modified BlalockTaussigThomas shuntsubclavian to pulmonary artery anastomosis, using a Gore-tex graft, to increase pulmonary blood flow
Potts shuntdescending aorta to left pulmonary artery anastomosis to increase pulmonary blood flow
Glenn shuntsuperior vena cava to right pulmonary artery anastomosis to provide low pressure pulmonary blood flow
Atrial switch repairsin both types of atrial switch repairs, the deoxygenated blood from the vena cavae goes through a baffle into
the left ventricle, which pumps the blood into the pulmonary artery. The oxygenated blood returning from the lungs passes through
the pulmonary veins via another baffle into the right ventricle that pumps the blood through the aorta into the systemic circulation.
Thus, the morphological right ventricle becomes a systemic ventricle and the morphological left ventricle, a subpulmonic ventricle.
Senning atrial switch repair for DTGAbaffles are created from the patients tissues (right atrial wall and part of the atrial septum)
Mustard atrial switch repair for DTGAbaffles are created from the pericardium and synthetic material
Waterston shuntascending aorta to right pulmonary artery anastomosis to increase pulmonary blood flow
Rastelli procedureright ventricle-to-pulmonary artery conduit to allow blood flow from the right ventricle to reach the branch
pulmonary arteries, usually in patients with right ventricular outflow tract obstruction
Rashkind procedurecatheter-based procedure for balloon atrial septostomy to allow intermixing of the deoxygenated right atrial
blood with the oxygenated left atrial blood in DTGA
Fontan (classic)right atrial to pulmonary artery anastomosis in tricuspid atresia
Jatenearterial switch for DTGA (anatomical correction with reimplantation of the coronaries)
Biventricular repairoperation to establish two functioning ventricles without the mixing of the venous and systemic circulations,
by closing the shunt defects and creating a connection between the right ventricle and the pulmonary circulation. Usually performed for tetralogy of Fallot, pulmonary atresia with VSD, transposition of the great arteries, and double-outlet ventricles.
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Determination of Atrioventricular
Relationship (the Location of Ventricles by
Bulboventricular Loop)
In the D-loop, the right ventricle is on the right while the
left ventricle is on the left. In the L-loop, there is ventricular
inversion with the morphological right ventricle on the
left and the morphological left ventricle on the right side.
Since the atrioventricular (AV) valves always follow their
respective ventricles, the tricuspid valve is attached to
the morphological right ventricle and the mitral valve is
attached to the morphological left ventricle. Accordingly,
the positions of the tricuspid and mitral valves are also
transposed in the L-loop. Other identifying features of the
AV valves are their positions and morphology. In the apical
four-chamber view, the position of the trileaflet tricuspid
valve is closer to the apex as compared with the mitral
valve. In the parasternal short-axis view, the mitral valve is
shaped like a fish mouth and has chordal attachments to
the two papillary muscles.
The crescent-shaped right ventricle is heavily
trabeculated as compared with the ellipsoid left ventricle.
The left ventricle usually has a smooth endocardial border
except in adults with left ventricular noncompaction or
in those with long-standing hypertension. In the right
ventricle, the presence of a moderator band (a prominent
muscle bundle crossing from the septum to the free wall),
and septal attachment of the tricuspid valve positioned
more apically that the mitral valve (since the AV valves are
always attached to their respective ventricles) provides
further confirmatory evidence.
Abdominal Situs
Abdominal situs is usually concordant with atrial situs.
Hence, it is often used to determine the atrial situs,
although the two follow each other around 70% of the
time. In atrial situs solitus, this relationship can be revealed
in the subcostal views by tracing the path of the inferior
vena cava, which is nonpulsatile (blue on color Doppler)
and located on the right of the spine as it passes through
the liver. The descending thoracic aorta is pulsatile (red on
color Doppler) and courses on the left of the spine. Gastric
folds and stomach bubble are also seen on the left side.
However, in atrial situs inversus, all these positions are
reversed with aorta to the right of the spine and the inferior
vena cava to the left of the spine. The subcostal view also
allows determination of direction along which the major
axis of the heart is aligned.
1796
Echocardiography in Pregnancy
The hemodynamic and physiological effects of pregnancy
in women with CHD pose an extra workload on the
cardiovascular system. As the pregnancy progresses into
the second and third trimesters, there is a 30% to 40%
increase in blood volume causing an 18% to 25% increase
in stroke volume. The heart rate rises by 20% and the
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Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
Echocardiography
Primarily, the subcostal, followed by the high parasternal
and apical four-chamber views are the best views in
assessment of the atrial septum. There is a likelihood
of a false-positive reading of an ASD due to midseptal
dropout in the apical four-chamber view. Pulsed and
color flow Doppler define the direction of the shunt most
accurately at a lower Nyquist level. The direction of the
low-velocity shunt depends on ventricular compliance.
Other low venous flows from the superior and the inferior
vena cava or the coronary sinus can cause a false-positive
signal suggestive of an interatrial communication by color
Doppler. A tricuspid regurgitant jet with the flow directed
toward the interatrial septum can also be misleading.
1799
Figs 75.5A to C: Diagram showing locations of the three common types of atrial septal defects (ASD): (A) Secundum ASD;
(B) Primum ASD; (C) Sinus venosus ASD. (LA: Left atrium; RA: Right atrium; RUPV: Right upper pulmonary vein; SVC: Superior vena
cava).
1800
Transesophageal Echocardiography
A TEE is necessary in nearly all adults for localizing/sizing
the secundum ASD, measuring septal rims (superior and
inferior), entry of the superior vena cava, connection of all
pulmonary veins, and for ruling out concomitant defects
before planning device closure or surgery. Color Doppler
can determine the major direction of the shunt (Fig. 75.6).
Measurements are made in orthogonal planes to allow
selection for appropriate candidates for device closures.
Three-dimensional TEE allows real time visualization of
the defect. It also allows more defined measurements of the
rims, size, and location with respect to adjacent structures,
and assessment of concomitant defects. In the current
era, it plays a major role in transcatheter implantation of
the ASD closure device.3236 TEE also helps in detecting
procedural complications such as device embolization
into the pulmonary artery or into the iliac artery.37,38 Early
and late complications such as erosions caused by devices
can also be demonstrated by TEE.39
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
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Contrast Echocardiography
An agitated saline contrast study is usually unnecessary
in adults with ASDs but when performed, it may reveal
a bidirectional shunt with the major component of a
Types
Secundummost common type of ASD
Primum ASDoften associated with an endocardial cushion defect/partial AV canal defect (inlet VSD) and cleft anterior mitral valve
Sinus venous ASDusually associated with partial anomalous pulmonary venous return
Coronary sinus ASDcommonly seen with persistent left superior vena cava
Common atrium
Associated defects
Occurs mostly as an isolated anomaly
Partial anomalous pulmonary venous return (sinus venosus defect)
May occur in association with the following defects:
Mitral valve prolapse
Ventricular septal defect
Patent ductus arteriosus
Pulmonary stenosis/right ventricular outflow tract obstruction
Tetralogy of Fallot
D-transposition of the great arteries
Congenitally corrected transposition of the great arteries
Truncus arteriosus
Tricuspid atresia
Ebsteins anomaly
Echocardiographic assessment
Identification of the type of defect
Estimation of the size of the defect in orthogonal views to get the largest dimension with 2D echocardiography or by planimetry
with 3D echocardiography
Color Doppler to assess to direction of the shunt (using low Nyquist limit)
Pulsed wave Dopplercharacteristic flow pattern begins in early systole, although most of the cardiac cycle with a broad peak in
late systole/early diastole, with peak velocity usually < 2 m/s (combination of pulmonary and systemic venous flow)
Right heart sizeright atrium and ventricle are enlarged when the shunt is significant with Qp/Qs over 1.5 (may avoid the need
to calculate Qp/Qs)
Estimation of right ventricular systolic pressure/degree of pulmonary hypertension
Postoperative
Rule out residual shunt
Device closure
Confirm alignment of the device along the interatrial septum
Rule out impingent on surrounding structuresmitral valve, aortic valve, and pulmonary veinsor erosion of the device
1802
Exercise Testing
Cardiac Catheterization
Diagnostic cardiac catheterization is not indicated in
younger adults with uncomplicated ASD if they have
had adequate noninvasive imaging. Shunt runs are of
value when the site of the defect is unclear, such as in
case of sinus venosus defect or a coronary sinus defect.
Cardiac catheterization is essential for assessment of
pulmonary vasoreactivity in adults with severe pulmonary
hypertension prior to ASD closure. In older adults,
coronary artery disease also needs to be ruled out before
surgical closure. Currently, cardiac catheterization is
mostly performed in conjunction with device closure of an
ASD.
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Type 4: Muscular
These VSDs may be multiple or large enough to persist
into adulthood. They are located in the trabecular septum
Echocardiography
Transthoracic echocardiography with color Doppler
displays location, size, and number of the VSD. For
calculation of pressure gradient across the VSD with
continuous wave Doppler, the cursor should be aligned
along the direction of the VSD for accurate assessment
of the shunt. Heart chamber dimensions, presence of
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
Cardiac Catheterization
It is usually performed in conjunction with device
closure of the defect. The major role of diagnostic
cardiac catheterization is in assessment of pulmonary
artery pressures and pulmonary vasoreactivity prior to
closure, when pulmonary hypertension is suspected on
echocardiography. In addition, coronary arteriography is
performed in older adults prior to surgical closure of the
defect.
VSD Closure
The Class I ACC/AHA recommendations for closure of a
VSD are the evidence of left ventricular volume overload
(Qp/Qs 2.0) or a history of infective endocarditis. VSD
closure is contraindicated in adults with severe irreversible
pulmonary vascular disease.
At other times, VSDs are closed during surgery for
aortic regurgitation and other associated defects.49
Although there is no Class I indication for catheterbased device closure of a VSD in an adult, the Class IIa
indications for device closure include high surgical risk
with hemodynamically significant residual shunt or
history of infective endocarditis. In these cases, the VSD
should not be located proximal to the aorta or the tricuspid
1805
Echocardiography
Echocardiography is sensitive and very specific in
diagnosing PDA. Tiny PDAs may sometimes be missed
and there is low likelihood of a false-positive PDA.50,51
Two-dimensional TTE is able to demonstrate a
persistent anatomical connection between the descending
aorta and the pulmonary artery only in some adults. This is
due to limited acoustic windows because of body habitus
or secondary to lower ultrasonic frequencies hampering
1806
Types
Perimembranousmost common
Supracristal/infundibular/subpulmonic
Muscular
Atrioventricular (AV) septal defect [inlet/atrioventricular (AV) canal defect]
Associated defects
Atrial septal defect
Patent ductus arteriosus
Coarctation of aorta
Aortic root dilatation
Aortic regurgitation
Tricuspid regurgitation
Echocardiographic assessment
Identification of the type of defect
2D and color Doppler evaluation of the size of defect in systole
Color and continuous wave Doppler to determine the direction and gradient of the shunt
Is it a restrictive or nonrestrictive VSD?
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1808
Transesophageal Echocardiography
In adults with limited acoustic windows, TEE is helpful in
assessment of the PDA and associated defects. It may reveal
more details such as the anatomy of ductus arteriosus and
its relationship with the descending aorta and pulmonary
artery. Real time 3D TEE helps in imaging of the PDA,
before and after Amplatzer device closure. However, the
cranial part of the aortic arch cannot be visualized because
of the near-field artifact.54
Live 3D TEE plays a major role in the perioperative
monitoring during transcatheter device closure by cutting
down the fluoroscopy time. It is useful in defining the
Cardiac Catheterization
Cardiac catheterization is primarily performed at the time
of device closure to determine the anatomy of the ductus,
degree of shunt, pulmonary vascular resistance (PVR),
pulmonary vasoreactivity, associated defects, and coronary
artery disease in older adults. Catheter-based device closure
is mainly guided by fluoroscopy and partially by TEE.
Associated defects
Usually occurs as an isolated anomaly
May be associated with the following defects:
Ventricular septal defect
Coarctation of aorta
Valvular defectsaortic or mitral valves
Complex CHDsin association with complex defects such as congenitally corrected transposition of the great arteries or tetralogy of Fallot.
Echocardiographic assessment
Identification of the defectwhen visible, the bifurcation of the main pulmonary artery may appear trifurcate with the ductus arising along with left and right pulmonary arteriesmeasure length and width when possible
Color Dopplerjet seen through the left pulmonary artery into the main pulmonary artery with marked spectral dispersion in
systole and diastole (throughout the cardiac cycle in adults) in a typical PDA with a left-to-right shunt
Assessment of pressure gradient across the PDA
Measurement of left ventricular dimensions and quantitative assessment of left ventricular functionleft heart enlargement is
common with moderate to large PDA
Estimation of right ventricular systolic pressures/pulmonary hypertension (right ventricular hypertrophy occurs in adults with
Eisenmenger physiology)
Rule out pulmonary artery dilatation, aneurysm, or calcification (often noted by the fourth decade of life)
Assessment of the aortic archrule out flow acceleration/obstruction, diastolic flow reversal
Postoperative
Left ventricular size and function, left atrial enlargement
Rule out residual shunts
Ensure proper device placement (a well-seated device can be visualized between the pulmonary artery bifurcation and the
inferior margin of the aorta. It should not protrude into the aortic lumen or the pulmonary artery)
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
1809
Echocardiography
PDA Closure
The ACC/AHA Class I recommendations3 for device or
surgical closure of PDA in adults are:
Left heart enlargement (atrial and/or ventricular) due
to volume overload or in the presence of pulmonary
hypertension due to a left-to-right shunt
History of endarteritis.
Surgical or device closure of PDA is contraindicated in
adults with severe pulmonary hypertension due to reversal
of the shunt (right to left). Calcification and tissue friability
in adults can make surgical closure challenging and
therefore, it is performed in limited cases. Surgery instead
of device closure should be considered in adults who have
calcified PDA, ductal aneurysm, history of endarteritis,
and very large PDAs.
Fig. 75.11: Diagrammatic representation of the transthoracic apical four-chamber view showing the distinctive feature of AV septal
defect with the bridging leaflets of the AV valves viewed in the
same horizontal plane (as shown with the dotted line). (LA: Left
atrium; LV: Left ventricle; MV: Mitral valve; PV: Pulmonary vein;
RA: Right atrium; RV: Right ventricle; TV: Tricuspid valve).
1810
Fig. 75.12: Transthoracic echocardiogram showing a cleft anterior mitral valve (MV) in the parasternal short-axis view. (AMVL:
Anterior mitral valve leaflet; PMVL: Posterior mitral valve leaflet).
Echocardiography
TTE with an agitated saline contrast study helps in
making the diagnosis. The agitated saline contrast
injection (bubble study) in the left arm via a peripheral
intravenous catheter flows into the left brachiocephalic
vein with appearance of microbubbles in the left atrium in
case of an unroofed coronary sinus. If the LSVC is draining
into an intact enlarged coronary sinus, an injection
into either the right or left arms (through a peripheral
intravenous catheter) will demonstrate the microbubbles
in the enlarged coronary sinus and then in the dilated
right heart.61 If the right superior vena cava is absent, the
injection of agitated saline contrast into the right arm will
first appear in the enlarged coronary sinus followed by the
right atrium.62
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Echocardiography
Two-dimensional echocardiography shows an aneurysmal sac, which can present as an abnormal, circular, thinwalled structure protruding into a heart chamber. Ruptured
aneurysms will lead to chamber enlargement over time.
Color Doppler with transthoracic echocardiogram will
show flow originating in the aorta and flowing into a
heart chamber (usually the right ventricle) in a ruptured
aneurysm. Multiple views, starting with the parasternal
long-axis and short-axis views at the level of the aortic
root, are required to locate the sinus from which the
aneurysm originated. The transducer is then angulated to
obtain modified views and locate the aneurysm (usually
above the aortic cusps). Contrast echocardiography allows
more precise definition of the aneurysmal sac. It aids in
the diagnosis of a left-to-right shunt by demonstrating
a negative contrast image in the right chambers with a
ruptured aneurysm and no negative contrast image with
an unruptured aneurysm.64 TEE delineates the anatomical
details such as the site of origin by displaying the aortic
root and sinuses more clearly. Three-dimensional
echocardiography adds incremental value by further
defining the spatial relationships between the aneurysm
and the cavity into which it drains.
Associated echocardiographic findings include aortic
regurgitation resulting from altered aortic root anatomy.
However, if there is severe aortic regurgitation, one
must rule out extensive damage to the aortic leaflets
by endocarditis or acute rupture of sinus of Valsalva
aneurysm. Chronic small fistulae with a left-to-right shunt
cause left heart enlargement or right atrial enlargement
when the fistula drains into the right atrium.
Echocardiography
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
Cardiac Catheterization
Cardiac catheterization is best tool for diagnosing and
tracing the entire course of the coronary artery. Coronary
angiography also allows accurate assessment of obstructive
coronary atherosclerosis is adults who are at higher
cardiovascular risk. While small coronary fistulae do not
require closure, the larger ones may require percutaneous
closure or surgery to avoid the risk for developing heart
failure or angina.
1813
VALVULAR DISEASE
Tricuspid Valve
Ebsteins Anomaly
First described by Wilhelm Ebstein in 1866, this rare
cyanotic CHD occurs in <1% of CHD with varying degree
of apparent apical displacement of the point of attachment
of the basal insertions of the septal and posterior leaflets of
the tricuspid valve.71 Due to failure of delamination, there
is adherence of tricuspid valve leaflets to the underlying
myocardium. In addition, there is apical displacement
of the functional tricuspid valve annulus with the septal
leaflets more apically displaced than the posterior,
followed by the anterior leaflets.72
The malformed tricuspid valve may be incompetent,
stenotic, or rarely, imperforate. If the right ventricle is
subdivided into the inlet, trabecular, and outlet portions,
then the displacement of the tricuspid orifice to the
junction of the inlet and trabecular ventricular zones
describes the essence of this anomaly.73
Due to atrialization of the right ventricle, by the
downward displacement of the functional tricuspid
annulus, the right atrium appears larger than its actual size.
The right ventricle, therefore, appears to be smaller and
may have impaired function over time. The left ventricular
function may also decrease in adulthood. The downward
displacement of the septal tricuspid valve leaflet is
associated with discontinuity of the central fibrous body
and septal atrioventricular ring, thus creating a potential
substrate for accessory atrioventricular connections and
ventricular pre-excitation, making the patient at risk for
sudden death. Right heart catheterization, therefore,
carries a risk of inducing ventricular arrhythmias leading to
sudden cardiac death. On angiography, morphofunctional
abnormalities of the left ventricle have been demonstrated
in many patients, which may be explained by increased
fibrosis in the left ventricular wall and the ventricular
septum as demonstrated by histological studies.74
The anterior tricuspid leaflet may be fenestrated
and redundant, giving it a sail-like appearance. The
septal leaflet may be tethered. The severity of tricuspid
regurgitation, presence of a PFO, or ASD determines
clinical outcomes. This defect is often associated with
pulmonary stenosis/atresia and VSDs, congenitally
corrected transposition of the great arteries, and tetralogy
of Fallot.31
1814
Echocardiography
In the current era, Ebsteins anomaly is most commonly
diagnosed by echocardiography.72 On transthoracic
echocardiography, the Ebsteins tricuspid valve is best
seen in the apical four-chamber view with its characteristic
sail-like elongated anterior leaflet and the downward
displacement of the septal leaflet caused by the tethering
to the septum, making it appear as though it is attached
apically in relation to the point of attachment of the anterior
mitral leaflet (Fig. 75.14). The diagnostic feature of Ebsteins
anomaly is an apical displacement of both the septal
and the posterior tricuspid leaflets, exceeding 20 mm or
8 mm/m2 in adults.75 In an apical four-chamber view, this
distance is measured from the point of attachment of the
Stress Echocardiography
Stress echocardiography with treadmill testing offers an
objective assessment of functional capacity, ventricular
contractile reserve, impact of exercise on arrhythmias, and
cyanosis in adults with Ebsteins anomaly.
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Associated defects
Tetralogy of Fallot
Echocardiographic assessment
Postoperative
Cardiac Catheterization
Coronary angiography is performed before surgical
intervention in patients at risk for coronary artery disease
or for assessment of pulmonary vasoreactivity in adults
with severe pulmonary hypertension.
1816
Pulmonary Valve
Pulmonary Stenosis
While the majority of the individuals have narrowing of the
pulmonary valve at the valvular level, stenosis may occur
at the subvalvular or supravalvular levels. Among the three
morphological types, the commonest one is the domeshaped pulmonary valvea mobile valve with narrow central
opening and systolic doming seen on echocardiography.
It is usually associated with main pulmonary artery
dilatation that results from structural abnormalities.11 The
three rudimentary raphe fuse to cause valve stenosis that
may increase in severity due to calcification. Over time,
pulmonary regurgitation may develop and the jet usually
directed toward the left pulmonary artery. Other associated
defects are ASD, VSD, and infundibular or right ventricular
hypertrophy leading to dynamic obstruction of the right
ventricular outflow tract.31
Dysplastic pulmonary valve has significant myxomatous thickening of the valve leaflets that reduces leaflet
mobility. Associated commonly with Noonan syndrome,
it may also manifest with narrowing of the pulmonary
annulus and the right ventricular outflow tract.
Unicuspid or bicuspid pulmonary valve may rarely be
seen in adults with tetralogy of Fallot. The quadricuspid
pulmonary valve has been reported as a rare finding in
literature, with a rudimentary accessory cusp interposed
between three cusps of same size in most cases. It may
present with stenosis/regurgitation and pulmonary artery
dilatation/aneurysm. The most common CHDs associated
with it are ASD, VSD, PDA, and bicuspid aortic valve.78
Echocardiography
The era of echocardiographic detection of the pulmonary
valve was heralded by Dr Nanda who published his
findings in 1972.77
On 2D transthoracic echocardiography, the parasternal
short-axis view at the level of the aorta is the best view for
assessment of the pulmonary valve morphology and
assessment of regurgitation or stenosis. The parasternal
long-axis view displays the right ventricular outflow
tract. The level and severity of the stenoses are important
in determining the timing for intervention or surgery.
Standard grading ranges from mild (peak valvular gradient
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Associated defects
Echocardiographic assessment
Valve annulus
Gradientdegree of stenosis
Postoperative
Valve gradientstenosis
Cardiac Catheterization
Cardiac catheterization is rarely necessary for diagnosis
and is usually performed when catheter-based intervention
is indicated. For confirmation, the gradients should be
obtained at the valvular, sub-, and supravalvular levels.
The presence of obstruction in the infundibulum as well
as main, branch, or peripheral pulmonary arteries should
be ruled out. It is important to note again that the peakto-peak gradient by cardiac catheterization correlates
best with the mean Doppler rather than with the peak
instantaneous Doppler gradient.78 Right ventriculogram is
performed for assessment of the right ventricular function
and degree of pulmonary regurgitation.
1818
Mitral Valve
Cor Triatriatum
Also known as cor triatriatum sinister, this is a very rare
cardiac defect that may go undiagnosed into adulthood
especially when it presents as an isolated anomaly. It
is characterized by a perforated membrane (partial or
complete) dividing the left atrium into two chambers
the proximal (pulmonary venous chamber) receives the
pulmonary veins, and the distal true chamber comprises
the left atrium with the fossa ovalis and left atrial
appendage.31 Associated defects include patent foramen
ovale, secundum ASD, and rarely a common atrium.
Adults usually present with symptoms similar to mitral
stenosis due to obstruction to flow through the perforated
Echocardiography
The best view is the apical four-chamber view that shows
a thin undulating membrane moving toward the mitral
valve in diastole and away from it in systole. The proximal
chamber is usually dilated due to a back up of flow
caused by obstruction through the perforated membrane
into the low pressure distal chamber. The left atrial
appendage is usually normal in size. Color flow Doppler
shows flow acceleration at the sites of perforations in the
membrane. The right ventricular systolic pressure should
be estimated from the tricuspid regurgitant jet to rule out
pulmonary hypertension.
It is important to establish the position of the left atrial
appendage in the distal chamber in order to confirm cor
triatriatum and exclude a supravalvular mitral ring.86
Transesophageal echocardiography is useful in
confirming the diagnosis by clearly delineating the
position of the left atrial appendage. It also allows complete
assessment of any associated intracardiac defects such as
the patent foramen ovale.83 Contrast echocardiography is
an important adjuvant technique where the perforation in
the membrane cannot be delineated by color Doppler. It
shows a differential opacification of the proximal versus
the distal chamber with delayed emptying of contrast into
the distal chamber through the communication between
the chambers.85 Three-dimensional reconstruction is
useful in diagnosing cor triatriatum by defining atrial
membranes and the associated defects.87
Although these echocardiographic modalities may
suffice, in some cases a left and right heart catheterization
with simultaneous pulmonary capillary wedge and left
ventricular end-diastolic pressure measurements may be
required to reveal a significant mean gradient, prior to
definitive surgical resection of the membrane.
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Echocardiography
Figs 75.16A and B: Transthoracic echocardiogram showing that the mitral valve area in an adult with a double orifice mitral valve (DOMV) is
calculated as the sum of the area of the two separate mitral orifices (A and B), in the parasternal short-axis views by planimetry.
1820
Transesophageal Echocardiography
TEE offers a more detailed analysis of the defect and
isolated defects in multiplane views. It often allows
differentiation between a true parachute and parachutelike mitral valve. In a parachute-like mitral valve, most
of the chordae tendinae are attached to a main papillary
muscle while the other papillary muscle is hypoplastic and
close to the main one.98 TEE should also be considered
when the severity of stenosis or regurgitation indicates
surgery in accordance with the ACC/AHA guidelines for
valvular disease.99
The ACC/AHA Class I recommendations for surgical
treatment for young adults with mitral valve disease are
the following:99
Congenital Mitral Stenosis:
Symptomatic young adult patients with congenital
mitral stenosis and NYHA functional Class III or IV,
who have a mean mitral valve gradient > 10 mm Hg on
Doppler echocardiography.
Other Class I indications that apply to all mitral valve
disorders include:
Symptomatic adults with NYHA functional Class
IIIIV, who have moderate or severe mitral stenosis
measured by other methods when percutaneous
mitral balloon valvotomy is either not available;
contraindicated because of concomitant moderate
to severe mitral regurgitation; persistent left atrial
thrombus despite anticoagulation; or unfavorable
mitral valve morphology.
Echocardiography
The transthoracic parasternal long-axis and shortaxis views (at the level of the papillary muscle), apical
four-chamber and two-chamber views are valuable
in assessment of the morphology of the mitral valve,
subvalvular apparatus, papillary muscle, and associated
left heart lesions. The parasternal long-axis view may show
chordae tendinae converging to the single papillary muscle
either as short, thickened, underdeveloped, and adherent
causing stenosis, versus elongated and floppy without
adequate coaptation of the mitral valve leaflets/prolapse
resulting in mitral regurgitation. In the parasternal shortaxis view at the midpapillary level, a single papillary is
visualized posteromedially and the mitral orifice may be
eccentric. In the parasternal short-axis view at the basal
level, parachute leaflets described as typical for this
condition are often seen.
Mitral Regurgitation
Mitral valve repair is preferred over MV replacement in
most cases:
Symptomatic adult with severe congenital mitral
regurgitation with NYHA functional Class III or IV
symptoms
Asymptomatic adult with severe congenital mitral
regurgitation and left ventricular ejection fraction
60%
Severe mitral regurgitation with NYHA functional
Class II, III, or IV symptoms in the absence of severely
reduced left ventricular function (ejection fraction
< 30%) and/or end-systolic dimension > 55 mm
Asymptomatic adult with chronic severe MR with
mildly to moderately reduced left ventricular function
(ejection fraction between 30% and 60%, and/or endsystolic dimension 40 mm).
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
Aortic Valve
Bicuspid Aortic Valve
Biscuspid aortic valve (BAV) is defined by the presence of
complete or partial fusion of two aortic leaflets, often manifesting as right and left leaflet fusion, otherwise as right
and noncoronary cusp fusion, or as left and noncoronary
cusp fusion.31 While right and left leaflet fusion is more
often associated with stenosis/regurgitation requiring
early intervention, the right leaflet and noncoronary
cusp fusion is more likely to be associated with aortic
root dilatation.100102 The cusp size may be unequal due to
fusion of two cusps leading to one larger cusp. A central
raphe may divide the larger of the two cusps at the site of
congenital fusion of two parts of the conjoined cusps.
Although many adults are previously diagnosed with a
bicuspid aortic valve because of a heart murmur, in others
the diagnosis is first made when they acquire endocarditis
and present with acute severe aortic regurgitation due
to aortic leaflet destruction or perforation, leading to
heart failure. Secondary infection of the mitral valve due
to contiguous spread of the infection is known to occur.
Destruction of the infected valves causes regurgitation
and the vegetations increase the risk of embolic stroke.
Sepsis is common in this scenario. Siniawski et al reported
high mortality (approximately 29%) in adults who have
endocarditis with aortic ring abscess and secondary
infection of the mitral valve requiring double valve surgery.
According to their study, the most potent independent risk
factors for mortality were septic shock and severe aortic
root destruction.103
First-degree relatives of patients with bicuspid
aortic valve should be screened by echocardiography
for the presence of a bicuspid aortic valve, since familial
reoccurrence of bicuspid aortic valve is approximately 9%,
mostly likely due to an autosomal dominant pattern of
inheritance with reduced penetrance.104
Severe calcification or regurgitation can be present in
third or fourth decade, often accelerated by risk factors
such as hyperlipidemia. The calcification and fibrosis
primarily occurs at the raphe and base of the cusps.105
Aortic regurgitation may result from inability of the
leaflets to coapt due to aortic root dilatation and loss of the
sinotubular junction, retraction of the leaflets caused by
fibrosis, prolapse of aortic cusps, aortic valve attempting
to close a perimembranous VSD (venturi effect), or
following endocarditis due to extensive valve destruction.
1821
Echocardiography
In the transthoracic parasternal long-axis view, doming
of the bicuspid aortic valve leaflets in systole can be
appreciated in most young adults who are unlikely to
have distorted dysplastic valve or restricted mobility due
to calcification of the valve. The parasternal short-axis
view (at the aortic valve level), is the best view for defining
valve anatomy by showing fusion between two cusps (one
smaller and one larger) or a variation of it. The extent of
commissural fusion determines degree of stenosis or
obstruction. In this view, the open bicuspid aortic valve
appears to look like an elliptical American football or
fish-mouth opening (Fig. 75.17).
Two-dimensional echocardiography and continuous
wave Doppler are routinely used to determine the severity
of valve stenosis using the same criteria as in the general
population, defined by the ACC/AHA valvular heart disease
guidelines.99 The peak aortic velocity should be assessed
by continuous wave by Doppler echocardiography in
multiple views and the highest values should be recorded
(usually best captured in the suprasternal views). The peak
and mean gradients are derived accordingly. Since the
1822
Cardiac Catheterization
Cardiac catheterization is recommended for assessment
of coronary arteries before aortic valve surgery in
adults who are at risk for coronary artery disease. It is
also recommended when a pulmonary autograft (Ross
operation) is being considered, so that the origin of the
coronary arteries can be defined in case CTA/MRI are not
being performed. It is of incremental value when there is
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
1823
Associated defects
Coarctation of aorta
Echocardiographic assessment
Valve annulus
Gradientdegree of stenosis
Postoperative
Stress Testing
Although stress testing with exercise may be performed
cautiously to assess functional capacity and blood
pressure response in asymptomatic adults who do not
have critical stenosis, it is contraindicated in symptomatic
adults, in those with repolarization abnormalities on ECG,
or systolic dysfunction on echocardiography.
Dobutamine stress testing can be performed
judiciously in adults with low-gradient aortic stenosis in
the setting of low left ventricular ejection fraction, since
the low cardiac output may mask a higher grade of
stenosis.
The ACC/AHA Class I indications for aortic valve
replacement are the following:3
1824
Aortic Stenosis:
Severe aortic stenosis or chronic severe regurgitation
in an adult undergoing coronary artery bypass graft
surgery or any other cardiac surgery of the aorta or
concomitant defect
Severe aortic stenosis with left ventricular function
< 50%
Symptomatic severe aortic regurgitation with left
ventricular ejection fraction < 50% and left ventricular
dilatation
Aortic root surgery is indicated when the ascending
aorta diameter is 5.0 cm or when it is increasing by
5 mm or more per year.
Aortic Regurgitation:
Symptomatic (angina, syncope, or dyspnea on
exertion) chronic severe aortic regurgitation
Asymptomatic adult with chronic severe aortic
regurgitation with reduced systolic function (ejection
fraction < 50% confirmed on two echocardiograms
(13 months apart)
Asymptomatic chronic severe aortic regurgitation with
progressive left ventricular enlargement (end-diastolic
dimension > 4 standard deviations above normal)
should receive aortic valve repair or replacement.
Subaortic Stenosis
Subaortic stenosis is a congenital obstruction below the
aortic valve that may present as a discrete shelf-like
fibrous ring or as a fibromuscular tunnel-type defect
causing a fixed left ventricular outflow tract obstruction
below the aortic valve.31
Echocardiography
Subaortic stenosis is best seen in the parasternal long-axis
view that delineates the left ventricular out flow tract with
the transducer positions perpendicular to the membrane.
The left ventricular outflow tract (LVOT) obstruction
is examined carefully with color flow Doppler, since
the obstruction may present at multiple levels. Aortic
regurgitation may occur due to long-standing subaortic
flow disturbance.
In most adults, 2D transthoracic echocardiography
defines the anatomy of the subaortic stenosis, the
dimensions of the ascending aorta, adjacent mitral valve
involvement, degree of left ventricular hypertrophy, and
function (both systolic and diastolic). Sometimes a thin,
wispy, discrete fibrous subaortic ring may not be clearly
visible on transthoracic echocardiography. Doppler helps
in estimating the degree of aortic valve regurgitation. The
severity of the gradient across the left ventricular outflow
tract and the aortic valve can be determined by continuous
wave Doppler (Fig. 75.18).
The location of a VSD has an impact in the estimating
of the severity of subaortic stenosis. The degree of
subaortic stenosis may be underestimated when the VSD
is proximal to subaortic obstruction and overestimated
when it is distal to it. As in the case of aortic stenosis,
reduced left ventricular systolic function will also lead
to underestimation of the gradient. Among the factors
affecting progression of the left ventricular outflow tract
obstruction are the position of the membrane adjacent to
the aortic valve and whether it extends toward the mitral
valve.
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
1825
Transesophageal Echocardiography
TEE is useful preoperatively and intraoperatively, in adults
with limited acoustic windows, to define the morphology
of the subaortic stenosis, and assess associated defects.
Intraoperative TEE evaluates adequate resection of the
subaortic membrane or ridge and rules out residual aortic
regurgitation. It also confirms the absence of iatrogenic
damage to the anterior mitral valve leaflet or creation of a
VSD by aggressive resection of the membrane. Similarly,
3D echocardiography may further define left ventricular
outflow anatomy.
Cardiac Catheterization
Preoperatively, cardiac catheterization is performed in
adults who are at high risk for coronary artery disease.
1826
Echocardiography
The left ventricular size, mass, systolic, and diastolic
function should be assessed on serial echocardiograms in
adults with supravalvular aortic stenosis. In most adults, the
parasternal long-axis view demonstrates the morphology
of the aortic root and allows measurement of the diameter
at the levels of the annulus, midsinus, sinotubular junction,
and the ascending aorta 2 cm distal to the sinotubular
junction. In others, the tubular portion of the aorta is not
well visualized and the best views are the suprasternal or
high right parasternal views. Increased continuous wave
Doppler gradient across an anatomically normal aortic
valve should alert the echocardiographer about a possible
obstruction. Pressure recovery phenomenon may be seen
in adults with long-segment or tubular stenosis.
Transesophageal echocardiography is useful in
obtaining this information in adults with limited acoustic
windows on transthoracic echocardiography. It also
defines the origins of the coronary arteries.
Stress Echocardiography
In adults presenting with symptoms and signs suggestive
of ischemia, stress echocardiography can be performed
to assess for coronary involvement and evaluation of the
gradient across the stenosis before and after exercise.
Cardiac Catheterization
For definitive diagnosis of coronary involvement and for
accurately measuring the gradient across the supravalvular
stenosis, angiography is the preferred diagnostic imaging
modality.
Coarctation of Aorta
Coarctation of aorta (COA) is discrete or segmental
narrowing of the aorta below the origin of the left
subclavian artery, at the junction of the distal aortic arch,
and the descending aorta (in most cases) or its variation.31
It is appears more likely to be a diffuse arteriopathy with
associated structural abnormalities of the great arterial
walls that are not just limited to focal stenosis. Hence,
it is no longer considered as a simple defect.118 The
commonest associated defects are a bicuspid aortic valve
followed by mitral valve disease. These individuals are also
prone to aortic root dilatation.
Prior to the era of surgeries or interventions, the
survival was only 50% by 32 years of age.119 While many
individuals are operated in childhood in the present
era, some are still diagnosed in adulthood during an
evaluation for secondary hypertension.120 Despite relief of
stenosis by surgery or catheter-based intervention, adults
have long-term residua, sequelae, increased morbidity,
and reduced life spans. The most common issue is
ambulatory hypertension and significant left ventricular
hypertrophy seen on an ECG or an echocardiogram.
Despite successful COA repair, recurrence at the site of
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
Echocardiography
Two-dimensional echocardiography is highly specific
in diagnosing aortic arch obstruction.124 The aortic
arch should be first interrogated carefully by 2D
echocardiography with pulsed Doppler recording, under
direct visualization. On transthoracic echocardiography,
the coarctation is best seen in the suprasternal or high right
parasternal views, also allowing visualization of the aortic
arch and proximal descending aorta. Presence of color
flow Doppler turbulence in the aortic arch brings to our
attention the maximum site of narrowing. Continuous
wave spectral Doppler interrogation is used to assess
the gradient. It shows characteristic flow profile with an
increased systolic flow velocity and continuous gradient
of forward diastolic flow. The more accurate formula
for estimating the pressure gradient is 4(V2-V1)2 with
V1 being the velocity proximal to the coarctation and V2
being the peak velocity at the site of maximal narrowing.
This formula avoids inaccurate reports of high pressure
gradients obtained by the simplified Bernoulli equation
(4V2) in patients with long tubular narrowing of the aortic
arch.125 A peak gradient of over 20 mm Hg indicates a
significant obstruction. However, the gradient may be
low or negligible if there are collaterals or ductal flow.
1827
Transesophageal Echocardiography
Agrawal et al. showed the incremental value of TEE in
delineating the aortic arch branches. Their protocol can
be adapted for the multiplane TEE probe.127 The short-axis
view of the proximal descending aorta is first identified as
a circular cavity in the transverse plane in the midthoracic
level. Further withdrawal of the probe brings the aortic
arch into view with the loss of the circular configuration
transitioning into a horizontal tube-like structure.
Switching from the transverse to the longitudinal plane (90)
demonstrates all three vessels (Fig. 75.1). The longitudinal
cut of the aortic arch makes it appear circular or ovoid.
Small movements of the probe (rotation, movement,
1828
Associated defects
Echocardiographic assessment
Postoperative
Rule out recoarctation or aneurysm at the site of the surgery (especially with patch repair)
Left ventricular hypertrophy (hypertension usually persists despite surgical relief of the coarctation)
Stress Echocardiography
Stress echocardiography with color and continuous wave
Doppler from the suprasternal notch allows assessment
of the coarctation gradient at rest and following exercise,
in addition to providing information obtained by the
treadmill stress testing. Coronary artery disease occurs
prematurely in this population, especially in those with
uncontrolled ambulatory hypertension.
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
Cardiac Catheterization
Coronary artery disease should be ruled out in adults prior
to surgery. Stent implantation is the preferred intervention
compared to balloon angioplasty alone for coarctation of
aorta in adults, with persistent relief of stenosis and lower
incidence of aneurysm formation.
1829
Tetralogy of Fallot
Four defects make the tetralogy of Fallot (TOF)a VSD, an
aorta that overrides the large ventricular septal defect (by <
50% of its diameter), subpulmonary infundibular stenosis,
and right ventricular hypertrophy. The presence of an ASD
would make it the pentalogy of Fallot!31
The clinical presentation is affected by the variable
degree of right ventricular outflow tract obstruction. The
most extreme form is pulmonary atresia with a VSD.
Associated defects are common and include abnormal
or absent pulmonary valve, pulmonary artery anomalies
(dilated or hypoplastic), aortic root dilation, aortic
regurgitation, right aortic arch (approximately 2030%),
and abnormal origin of the coronary (left anterior
descending coronary artery arising from the right coronary
artery and crossing the right ventricular outflow tract).
Most adults have had previous palliative shunts or
complete intracardiac repair but continue to present
with multiple residua and sequelae. The intracardiac
repair comprises closure of the VSD with redirection of
the aorta toward the left ventricle, and resection of the
right ventricular outflow tract obstruction with relief of
hypertrophied muscle that is contributing to infundibular
stenosis. Following an intracardiac repair of TOF, the most
common long-term cardiac issue is chronic pulmonary
valve regurgitation that requires reoperations (pulmonary
valve replacement), approximately every 10 to 20 years.
1830
Echocardiography
A comprehensive transthoracic echocardiographic evaluation in an adult with tetralogy of Fallot postintracardiac
repair begins with the assessment of the right and left
ventricular size and function. Left ventricular function
is estimated by standard methods. Besides the standard
measures for assessing left ventricular function, a
Doppler-derived index of LV filling pressure (the ratio
of early transmitral flow velocity to early diastolic mitral
annular velocity) is a powerful predictor of ventricular
arrhythmias.142
Right ventricular enlargement and declining systolic/
diastolic function are common especially in the setting
of progressive pulmonary regurgitation. The enlarging
right ventricle stretches the tricuspid annulus causing
progressive tricuspid regurgitation, followed by right atrial
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
1831
Right ventricular diastolic function: Isolated right ventricular restriction late after tetralogy of Fallot repair is fairly
common. Methods for assessment of right ventricular
diastolic function and restrictive physiology have been of
major research interest. The clinical applications of these
time-consuming protocols have been limited so far due
to difficulty in accurately using them on the distorted
right ventricular anatomy. Gatzoulis et al. showed that
antegrade diastolic flow in the main pulmonary artery,
coinciding with atrial systole (A-wave) throughout the
respiratory cycle was indicative of right ventricular
restriction. On pulsed Doppler sampling (at the midpoint
between the pulmonary valve leaflets and bifurcation of
the main pulmonary artery), the duration of pulmonary
regurgitation was noted to be shorter in those with right
ventricular restriction. This finding was thought to be
reflective of reduced right ventricular diastolic compliance,
with restrictive physiology at end-diastole, thereby making
the right ventricle a passive conduit between right atrium
and pulmonary artery during atrial systole.150 However, it
can also be present in normal hearts during inspiration.
Therefore, it should be recorded for at least five consecutive
beats to be of diagnostic value in identifying reduced right
ventricular diastolic compliance.
Other features on pulsed Doppler that support restrictive
physiology include: Superior vena caval flow reversal
with atrial systole (measured 12 cm proximal to the
right atrium), and shorter inspiratory and expiratory
transtricuspid E-wave deceleration times (measured at the
level of the tricuspid valve leaflets).
1832
Stress Echocardiography
Exercise testing is required for objective assessment
of functional capacity and to unveil exercise-induced
arrhythmias such as atrial fibrillation/flutter, premature
ventricular complexes, and nonsustained ventricular
tachycardia in the postoperative adult with tetralogy of
Fallot. Monomorphic ventricular tachycardia most often
occurs in the presence of an old ventriculotomy scar. Poor
heart rate response to exercise unmasks chronotropic
incompetence that may lead to pacemaker implantation.
Stress echocardiography provides additional information regarding the severity of pulmonary hypertension
postexercise and the impact of pulmonary regurgitation
on the right ventricular contractile reserve, by gauging
its hyperdynamic response to exercise. Meijboom et al.
showed that exercise capacity inversely correlates with right
ventricular dilation in patients with repaired tetralogy.153
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
1833
Associated defects
Pulmonary stenosis
Pulmonary atresia
AV septal defects
Anomalous origin of the coronary arteries (left anterior descending arising from right coronary cusp and courses across the
right ventricular outflow tract)
Aortic regurgitation
Subaortic stenosis
Aortopulmonary window
Aortopulmonary collaterals
Anomalous coronaries
Echocardiographic assessment
Pulmonary regurgitation
Aortic regurgitation
Shunt lesionsventricular septal defects, atrial septal defect, patent ductus arteriosus, persistent left superior vena cava
Postoperative
Pulmonary regurgitation
Pulmonary stenosis
Aortic regurgitation
1834
A. Based on the Color Doppler width of the jet on the ventricular aspect of pulmonary valve leaflets
Tracea flash of color
Mildjet width < 20% of the annular width of the valve/conduit
Moderatejet width between 20% and 40% of the annular width of the valve/conduit
Severejet width > 40% of the valve/conduit annular width
B.
Cardiac Catheterization
Angiography plays a limited role in the current era for
the postoperative adult with tetralogy of Fallot. It is not a
reliable test for assessment of pulmonary regurgitation,
since the angiographic severity may be influenced by
catheter position across the pulmonary artery. It accurately
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
1835
1836
the aorta and pulmonary artery. This implies that the aorta
arises from the systemic morphological right ventricle
and lies rightward and anterior to the pulmonary artery,
which in turn arises from the pulmonic morphological left
ventricle.
Common associated defects include a shunt lesion, a
VSD (45%), an ASD, or a PDA that allows intermixing of the
deoxygenated and oxygenated blood to permit survival,
since this circulation is a parallel circuit and will not be
compatible with life without any communication. Most
individuals are diagnosed early in life with this form of
cyanotic congenital heart disease and have undergone one
of the types of repairs. Other associated defects include left
ventricular outflow tract (LVOT) obstruction, coarctation
of the aorta, and anomalies of the coronary ostia due to
transposition.31
Echocardiography
Serial echocardiography is performed in adults who have
undergone atrial switch repair to assess the morphological
right ventricular size and function. Most adults have a
globular-shaped, hypertrophied morphological right
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
1837
Associated defects
Shunt lesionsventricular septal defect (usually perimembranous), atrial septal defect and patent ductus arteriosus
Left ventricular outflow tract (LVOT) obstructionpulmonary stenosis, usually valvular but may be subvalvular
Echocardiographic assessment
Most of the adults have had surgeries in their childhood
Great arteriesaorta and pulmonary arteryare seen parallel to each other in the parasternal long-axis view.
In the short-axis view they are seen next to each other with the aorta being anterior and rightward.
Postoperative
Atrial switch repair
Baffle leak
Baffle obstruction
Coronary artery fibrosis may lead to ischemia/infarctionstress echocardiography is performed to assess wall motion abnormalities
Post-Rastelli repair
Conduit obstruction
1838
Stress Echocardiography
Exercise capacity may be limited postatrial switch
repair due to stiffness and noncompliance of the baffles,
deterioration in systemic ventricular function, sick
sinus syndrome, or advanced conduction disease. Baffle
problems appear to have the most effect on exercise ability
because of impaired ventricular filling and limited ability
to augment stroke volume during exercise.173 Hence,
functional capacity assessed objectively by exercise stress
echocardiography should be followed by a search for causes
of hemodynamic impairment such as baffle obstruction,
progressive tricuspid regurgitation, subpulmonic obstruction, impaired systemic ventricular function, or
chronotropic incompetence. Routine repetition of exercise
studies every 3 years is recommended even in the absence
of symptoms, since gradual declines in function often go
unnoticed by patients.169
Cardiac Catheterization
Systemic or pulmonary venous baffle obstructions are
diagnosed by angiography and can be often be relieved by
balloon-expanded stent implantation. Some of the baffle
leaks can be eliminated by implanting smaller devices.
Intracardiac echocardiography plays a role in guiding
percutaneous closure of atrial baffle defects.175
Echocardiography
Neoaortic dilatation and aortic regurgitation may develop
over time. Neoaortic constriction can occur at the site of
anastomosis. Patients with previous pulmonary artery
banding (although it is now rarely performed) are more
likely to have neoaortic root dilatation but it may not
necessarily progress on late follow-up. Risk factors for
aortic regurgitation include older age at the time of
presentation, presence of a VSD, and previous pulmonary
artery banding.177
Stress Echocardiography
Following atrial switch repair, stress echocardiography is
performed to screen for exercise-induced ischemia.
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
Cardiac Catheterization
Coronary ostial fibrosis leading to coronary ischemia
prompts early catheterization in those adults who have
undergone reimplantation of the coronaries into the base
of the neoaorta.
Post-Rastelli Repair
The Rastelli procedure is performed in d-TGA that is
associated with a large VSD and significant pulmonary
stenosis. In this surgical repair, a right ventricle to
pulmonary artery conduit allows blood flow from the right
ventricle to reach the branch pulmonary arteries, usually in
patients with right ventricular outflow tract obstruction.178
Echocardiography
Echocardiography plays an important role in evaluation
of long-term complications including right ventricular
outflow tract (RVOT) obstruction, pulmonary conduit
obstruction with degeneration and calcification of the
pulmonary valve, residual shunts, and degree of pulmonary
and tricuspid regurgitation. The tricuspid regurgitant jet
velocity helps in estimation of the right ventricular systolic
1839
Cardiac Catheterization
Cardiac catheterization determines the conduit gradient
accurately. Catheter-based interventions such as intraconduit percutaneous pulmonary valve implantation, and
dilation with or without stent implantation for relief of
branch pulmonary artery stenosis can be performed when
indicated.
1840
Echocardiography
Unfortunately, this diagnosis may be missed on echocardiography because of failure to identify the abnormal
position of the AV valves associated with ventricular
inversion. In addition, the 1% of adults with CCTGA without
associated defects are even more likely to go undiagnosed
into adulthood. Identifying the relationship of the great
arteries and defining the position of the morphological
tricuspid valve are essential for clinching the diagnosis on
echocardiography.
As seen in d-TGA, the two great arteries are parallel to
each other in the modified parasternal long-axis view. They
are seen in the same cross-sectional plane in the parasternal
short-axis views. The position of aorta is anterior, leftward,
and superior, while the pulmonary artery is rightward,
posterior, and inferior. Identification of the ventricular
morphology and positions of the AV valve that are best seen
in the apical four-chamber view remains critical in making
the diagnosis (Fig. 75.18). The AV valves always follow
their ventricles and, therefore, the tricuspid valve (referred
to as the systemic AV valve) is on the left side with the
morphological right (systemic) ventricle posing as the left
AV valve, while the mitral valve (referred to as the pulmonic
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
1841
Fig. 75.21: Transthoracic echocardiogram in congenitally corrected transposition of the great arteries (CCTGA) showing ventricular
inversion and tricuspid valve on the left side (apically placed in
relation to the mitral valve). (LA: Left atrium; LV: Left ventricle;
MB: Moderator band; MV: Mitral valve; RA: Right atrium; RV: Right
ventricle; TV: Tricuspid valve).
Source: With permission from Vijayalakshmi IB, Rao PS, Chugh
R, editors. A Comprehensive Approach to Congenital Heart Diseases. 1st ed. New Delhi: Jaypee Brothers Medical; 2013: 767.
Fig. 75.22: Transthoracic echocardiogram in congenitally corrected transposition of the great arteries (CCTGAs) showing that the
left ventricle pumps into the transposed pulmonary artery and
its branches (arrows) in a modified four-chamber view. (LV: Left
ventricle; RV: Right ventricle).
Cardiac Catheterization
The role of cardiac catheterization is mainly for delineating
the coronary anomalies or atherosclerosis prior to surgery.
It also allows evaluation of pulmonary vascular reactivity
in adults with significant pulmonary hypertension.
1842
Truncus Arteriosus
Associated defects
Tricuspid (left AV valve) anomalyEbsteins anomaly, Ebstein-like or dysplastic morphological tricuspid valve
Aortic regurgitation
Coarctation of aorta
Dextrocardia
Echocardiographic assessment
Postoperative
Aortic regurgitation
Thromboembolic risk
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
Associated defects
Echocardiographic assessmentpostoperative
1843
Echocardiography
On transthoracic echocardiography, the parasternal longaxis view shows the truncal root, valve, and sometimes the
origins of the pulmonary arteries. The apical four-chamber
view is helpful in demonstrating a VSD and an overriding
truncus. The suprasternal views allow identification of a right
aortic arch and coarctation of the aorta. Color Doppler is
useful in identifying truncal valve stenosis or regurgitation,
presence of pulmonary stenosis at the origin of the branches,
and for determining the direction of the shunt. An agitated
saline contrast study is performed to rule out right-to-left
shunts and to define the right ventricular outflow tract in
the parasternal short-axis views or the subcostal views.190,191
Associated defects are coarctation of aorta, interrupted aortic
arch, right aortic arch, patent ductus arteriosus, ASD, and
tricuspid stenosis. In the absence of significant pulmonary
stenosis, pulmonary hypertension develops. A synopsis
of echocardiographic assessment of truncus arteriosus is
reviewed in Table 75.18.
1844
Cardiac Catheterization
Besides evaluation of the coronaries, cardiac catheterization is performed in conjunction with stent placement
when there is conduit obstruction.
Echocardiography
Most individuals with DORV are operated in childhood.
Echocardiography in an unoperated adult focuses on the
relation of the aorta and pulmonary artery, the position
and relation of the VSD, any obstruction (subpulmonic
or subaortic), presence of pulmonary hypertension, and
identification of a constellation of associated defects.
Irreversible pulmonary vascular disease with Eisenmenger
physiology will preclude surgical repair in adulthood, as
seen in adults with large subaortic or subpulmonic VSDs
without pulmonary stenosis.199 The best views are the
parasternal and subcostal views for identification of the
origin of both great arteries from the morphological right
ventricle.
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
Stress Echocardiography
Stress echocardiography with treadmill stress testing
is valuable in assessing exercise intolerance related to
residual hemodynamically significant lesions, exerciseinduced arrhythmias, and chronotropic incompetency.
Cardiac Catheterization
In limited cases, cardiac catheterization is performed to
assess the pulmonary vascular resistance, and hemodynamic
status prior to repair and before transcatheter pulmonary
valve implantation in the patients with RV to PA conduits.
1845
Univentricular Heart
The spectrum of univentricular hearts comprises multiple
defects that are not amenable to biventricular repair and
rely on the dominant ventricle to support both the systemic
and pulmonary circulations. CHDs that present with the
single ventricle physiology are tricuspid atresia, mitral
atresia, hypoplastic right or left ventricle, and double inlet
left ventricle. The clinical manifestations and prognosis for
an adult with any form of a univentricular heart are related
to the pulmonary artery blood flow/pressure, which is
affected by the presence and degree of pulmonary stenosis,
and the level of pulmonary vascular resistance. The
majority of the adult survivors have undergone palliative
repairs: systemic to pulmonary artery shunt and/or Glenn
procedure, or Fontan procedure.
1846
Echocardiography
Echocardiographic assessment of the post-Fontan adult
looks into the important determinants that have a longterm effect on the systemic ventricular size and function.
The tricuspid atresia is best seen in the apical fourchamber view (Fig. 75.24). These include systemic AV
valve morphology, degree of valvular regurgitation, and
the presence of subaortic stenosis.
The cavoatrial, atrial septal, or ventricular septal
defects should be identified, since they contribute to the
persistence of cyanosis. In some individuals, fenestrated
atrial septum or an adjustable ASD are a necessity. In these
cases, the gradient across the fenestration or shunt should
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
Transesophageal Echocardiography
The onset of heart failure symptoms with hepatic
congestion and jugular venous distension (in those
without a Glenn shunt) or the development of new atrial
intractable arrhythmias prompts a search for conduit
stenosis. Conduit obstruction may be partially assessable
1847
Cardiac Catheterization
The etiology of oxygen-unresponsive hypoxemia in adults
with Fontan is determined by cardiac catheterization that
can identify potential causes including persistent Fontan
fenestration, systemic venous-to-pulmonary venous
collaterals, and pulmonary arteriovenous malformations.
In heart failure patients, the cause of volume retention
may be worsening ventricular function, increasing Fontan
circuit pressure, and resistance that exaggerate the rightto-left shunts. Catheter-based closure of residual shunts by
coils or ASD devices may relieve the symptoms.
Cardiac catheterization plays an important role in
assessment of protein-losing enteropathy (PLE) in the postFontan adult. Causes of increased resistance to effective
pulmonary flow, such as obstruction to pulmonary
flow at the pulmonary artery or venous levels, AV valve
stenosis or regurgitation may contribute to progressive
PLE. The aortic-pulmonary collaterals can be also be
1848
Associated defects
Echocardiographic assessmentpostoperative
Conduit obstructionobstruction of the Fontan connectionusually due to right pulmonary vein compression from an
enlarged right atrium or from conduit obstruction.
CONCLUSIONS
Echocardiography plays a vital role in diagnosis and
follow-up of adults with congenital heart defects. Every
effort should be made to acquire the previous medical
records, especially operation notes and imaging studies,
in order to understand the blueprint of each individuals
unique anatomy and physiology. It is essential to perform
a complete examination and be flexible in performing
modified views to acquire the desired images. Equally
important is our understanding about the indications for
applying the appropriate imaging modalities. Since our
eyes see what the mind knows, an echocardiographer
must have knowledge of the underlying anatomy, types
of surgeries, associated defects, long-term residua, and
sequelae in order to provide a comprehensive assessment
that will direct timely and appropriate treatment.
ACKNOWLEDGMENTS
My deepest gratitude to Dr John S Child and Dr Navin C
Nanda for their inspiration and the gift of knowledge; to
Robert Reber for his expertise as an audiovisual engineer;
to Ms Hovey Lee for the extensive literature searches;
to the patients and sonographers (Paul Junkel, Terri
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2. Ayres NA, Miller-Hance W, Fyfe DA, et al; Pediatric
Council of the American Society of the Echocardiography.
Indications and guidelines for performance of
transesophageal echocardiography in the patient with
pediatric acquired or congenital heart disease: report from
the task force of the Pediatric Council of the American
Society of Echocardiography. J Am Soc Echocardiogr.
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3. Warnes CA, Williams RG, Bashore TM, et al. ACC/AHA 2008
guidelines for the management of adults with congenital
heart disease. J Am Coll Cardiol. 2008;52(23):e143263. doi:
10.1016/j.jacc.2008.10.001
4. Chang RK, Alejos JC, Atkinson D, et al. Bubble contrast
echocardiography in detecting pulmonary arteriovenous
shunting in children with univentricular heart after
cavopulmonary anastomosis. J Am Coll Cardiol.
1999;33(7):20528.
5. Van Hare GF, Silverman NH. Contrast two-dimensional
echocardiography in congenital heart disease: techniques,
indications and clinical utility. J Am Coll Cardiol.
1989;13(3):67386.
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
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48. Jacobs JP, Burke RP, Quintessenza JA, et al. Congenital Heart
Surgery Nomenclature and Database Project: ventricular
septal defect. Ann Thorac Surg. 2000;69(4 Suppl):S2535.
49. Mongeon FP, Burkhart HM, Ammash NM, et al. Indications
and outcomes of surgical closure of ventricular septal
defect in adults. JACC Cardiovasc Interv. 2010;3(3):2907.
50. Houston AB, Gnanapragasam JP, Lim MK, et al. Doppler
ultrasound and the silent ductus arteriosus. Br Heart J.
1991;65(2):979.
51. Gutgesell HP, Huhta JC, Latson LA, et al. Accuracy of
two-dimensional echocardiography in the diagnosis of
congenital heart disease. Am J Cardiol. 1985;55(5):51418.
52. Prez JE, Nordlicht SM, Geltman EM. Patent ductus
arteriosus in adults: diagnosis by suprasternal and
parasternal pulsed Doppler echocardiography. Am J
Cardiol. 1984;53(10):14735.
53. Waggoner AD, Barzilai B, Prez JE. Saline contrast
enhancement of tricuspid regurgitant jets detected by
Doppler color flow imaging. Am J Cardiol. 1990;65(20):
136871.
54. Marek T, Zelizko M, Kautzner J. Images in cardiovascular
medicine. Real time 3-dimensional transesophageal
echocardiography imaging: adult patent ductus arteriosus
before and after transcatheter closure. Circulation.
2009;120(12):e923.
55. Sinha A, Nanda NC, Khanna D, et al. Live three-dimensional
transthoracic echocardiographic delineation of patent
ductus arteriosus. Echocardiography. 2004;21(5):4438.
56. Chuang YC, Yin WH, Hsiung MC, et al. Successful
transcatheter closure of a residual patent ductus arteriosus
with complex anatomy after surgical ligation using an
Amplatzer ductal occluder guided by live three-dimensional
transesophageal echocardiography. Echocardiography.
2011;28(5):E1013. doi: 10.1111/j.1540-8175.2010.01343.x.
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57. Allwork SP. Anatomical-embryological correlates in
atrioventricular septal defect. Br Heart J. 1982;47(5):
41929.
58. Singh A, Romp RL, Nanda NC, et al. Usefulness of live/real
time three-dimensional transthoracic echocardiography
in the assessment of atrioventricular septal defects.
Echocardiography. 2006;23(7):598608.
59. Singh P, Mehta A, Nanda NC. Live/real time threedimensional transthoracic echocardiographic findings
in an adult with complete atrioventricular septal defect.
Echocardiography. 2010;27(1):8790.
60. Gonzalez-Juanatey C, Testa A, Vidan J, et al. Persistent
left superior vena cava draining into the coronary sinus:
report of 10 cases and literature review. Clin Cardiol.
2004;27(9):51518.
61. Kliger C, Jelnin V, Perk G, et al. Use of multi-modality
imaging in a patient with a persistent left superior vena
cava, partial anomalous pulmonary venous connection,
and sinus venosus-type atrial septal defect. Eur Heart J
Cardiovasc Imaging. 2012;13(6):499.
62. Yuce M, Kizilkan N, Kus E, et al. Giant coronary sinus and
absent right superior vena cava. VASA. 2011;40(1):657.
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94. Shone JD, Sellers RD, Anderson RC, et al. The developmental
complex of parachute mitral valve, supravalvular ring of
left atrium, subaortic stenosis, and coarctation of aorta. Am
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95. Bolling SF, Iannettoni MD, Dick M 2nd, et al. Shones
anomaly: operative results and late outcome. Ann Thorac
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96. Schaverien MV, Freedom RM, McCrindle BW. Independent
factors associated with outcomes of parachute mitral valve
in 84 patients. Circulation. 2004;109(19):230913.
97. Espinola-Zavaleta N, Chugh R, Ramrez GM. Parachute
mitral valve with severe mitral regurgitation in an adult
patient. Echocardiography. 2012;29(5):E1225. doi:
10.1111/j.1540-8175.2011.01620.x. Epub 2012 Feb 14.
98. Hakim FA, Kendall CB, Alharthi M, et al. Parachute mitral
valve in adults-a systematic overview. Echocardiography.
2010;27(5):5816.
99. Bonow RO, Carabello BA, Chatterjee K, et al. 2006 Writing
Committee Members; American College of Cardiology/
American Heart Association Task Force. 2008 Focused
update incorporated into the ACC/AHA 2006 guidelines
for the management of patients with valvular heart
disease: a report of the American College of Cardiology/
American Heart Association Task Force on Practice
Guidelines (Writing Committee to Revise the 1998
Guidelines for the Management of Patients With Valvular
Heart Disease): endorsed by the Society of Cardiovascular
Anesthesiologists, Society for Cardiovascular Angiography
and Interventions, and Society of Thoracic Surgeons.
Circulation. 2008;118(15):e523661.
100. Fernandes SM, Sanders SP, Khairy P, et al. Morphology of
bicuspid aortic valve in children and adolescents. J Am
Coll Cardiol. 2004;44(8):164851.
101. Fernandes SM, Khairy P, Sanders SP, et al. Bicuspid aortic
valve morphology and interventions in the young. J Am
Coll Cardiol. 2007;49(22):221114.
102. Schaefer BM, Lewin MB, Stout KK, et al. Usefulness of
bicuspid aortic valve phenotype to predict elastic properties
of the ascending aorta. Am J Cardiol. 2007;99(5):68690.
103. Siniawski H, Grauhan O, Hofmann M, et al. Aortic root
abscess and secondary infective mitral valve disease: results
of surgical endocarditis treatment. Eur J Cardiothorac Surg.
2005;27(3):43440.
104. Braverman AC, Gven H, Beardslee MA, et al. The bicuspid
aortic valve. Curr Probl Cardiol. 2005;30(9):470522.
105. Roberts WC. The congenitally bicuspid aortic valve. A
study of 85 autopsy cases. Am J Cardiol. 1970;26(1):7283.
106. Larson EW, Edwards WD. Risk factors for aortic dissection:
a necropsy study of 161 cases. Am J Cardiol. 1984;53(6):
84955.
107. Zoghbi WA, Enriquez-Sarano M, Foster E, et al; American
Society of Echocardiography. Recommendations for
evaluation of the severity of native valvular regurgitation
with two-dimensional and Doppler echocardiography.
J Am Soc Echocardiogr. 2003;16(7):777802.
108. Keane MG, Wiegers SE, Plappert T, et al. Bicuspid aortic
valves are associated with aortic dilatation out of proportion
to coexistent valvular lesions. Circulation. 2000;102(19
Suppl 3):III359.
Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
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Chapter 75: Echocardiography in the Evaluation of Adults with Congenital Heart Disease
1855
CHAPTER 76
Echocardiographic Evaluation for
Acquired Heart Diseases in Childhood
Jie Sun, Rula Balluz, Lindsay Rogers, Shuping Ge
Snapshot
Infective Endocarditis
Endocarditis
Echocardiographic Findings
INTRODUCTION
As with congenital heart disease, acquired heart diseases are
also common in children. The spectrum includes infective
endocarditis, rheumatic heart disease (RHD), Kawasaki
disease, and other cardiovascular involvement related
to other systems or organs, such as hypertension, sickle
cell disease, chronic renal disease, and cancer survivors.
Echocardiographic diagnosis and assessment have become
a routine practice to evaluate these patients initially and
possibly longitudinally to facilitate diagnosis, prognosis, and
guidance for treatment. In this chapter, we will discuss three
of the most common acquired cardiovascular diseases in
children, namely, infective endocarditis, RHD, and Kawasaki
disease, and further in-depth discussion can be found in
comprehensive texts and other sources.
INFECTIVE ENDOCARDITIS
Infective endocarditis (IE) is an uncommon but lifethreatening infection. Despite advances in diagnosis,
antimicrobial therapy, surgical techniques, and manage
ment of complications, patients with IE still have high
morbidity and mortality rates related to this condition.
Kawasaki Disease
1857
ECHOCARDIOGRAPHIC FINDINGS
Echocardiography should be performed in any patient sus
pected of having IE to allow earlier diagnosis, treatment,
1858
Vegetation Location
The morphology of IE vegetations is dependent on the
location of the endothelial lesion and always follows the
pathological bloodstream. When IE occurs in association
with ventricular septal defect, the vegetation is typically
visualized on the right ventricular aspect of the septum
and/or on the site where the high-velocity jet strikes the
right ventricular free wall. In the case of patent ductus
arteriosus, the vegetation may float through the pulmonary
artery. In patients with regurgitation of atrioventricular
valves, the vegetation is located on the atrial side
COMPLICATIONS OF INFECTIVE
ENDOCARDITIS
In addition to its role in diagnosing IE, echocardiography is
important for recognizing the intracardiac complications
associated with IE including regurgitant valve lesions,
chordal rupture, valve perforation, prosthetic dehiscence,
and paravalvular leak. Vegetations may also extend to
the outside of valve into surrounding structures to cause
abscess, fistula, or pseudoaneurysm formation.
Negative Result
The implications of an initial TEE examination that fails
to show vegetations in a patient with suspected IE should
be carefully considered. Although TEE cannot definitively
rule out IE, its high diagnostic sensitivity results in a
low probability of the disease when negative results are
obtained in a patient with an intermediate likelihood of
the disease. However, repeat examination is important,
particularly in patients at high risk for IE. Although the rate
of false-negative TEE examinations in patients with IE is
low, negative results confer an obligation of careful followup with exercise of sound clinical judgment.
1859
Major Criteria
Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
Prophylaxis is reasonable because endothelialization of prosthetic material occurs within 6 months after the procedure.
1860
Minor Criteria
Arthralgia
Fever
Elevated acute phase reactants
Erythrocyte sedimentation rate
C-reactive protein
Prolonged PR interval
Plus supporting evidence of an antecedent group A
streptococcal infection:
Positive throat culture or rapid strep test
Elevated or rising streptococcal antibody titers
Acute Valvulitis
KAWASAKI DISEASE
Kawasaki disease is an acute, self-limited vasculitis that
occurs predominantly in infants and young children. It
was first described in Japan in l967 by Tomisaku Kawasaki.
The disease is now known to occur in both endemic and
community-wide epidemic forms in the Americas, Europe,
and Asia in all races. Kawasaki disease is characterized
by fever, bilateral nonexudative conjunctivitis, erythema
of the lips and oral mucosa, changes in the extremities,
rash, and cervical lymphadenopathy. Coronary artery
aneurysms or ectasia develop in approximately 1525%
of untreated children with the disease and may lead to
myocardial infarction, sudden death, or ischemic heart
disease.1820
In the United States, Kawasaki disease is the leading
cause of acquired heart disease in children. Treatment of
Kawasaki disease in the acute phase is directed at reducing
inflammation in the coronary artery wall and preventing
coronary thrombosis. The long-term therapy in individuals
who develop coronary aneurysms is aimed at preventing
myocardial ischemia or infarction.
The etiology of Kawasaki disease remains unknown,
although clinical and epidemiological features strongly
suggest an infectious cause. It also is possible that Kawasaki
disease results from an immunological response that is
triggered by any of several different microbial agents.
Kawasaki disease is a generalized systemic vasculitis
involving blood vessels throughout the body. Aneurysms
may occur in other extraparenchymal muscular arteries.
The early stages in the formation and development of
arteritis in Kawasaki disease have been well studied
morphologically in relatively large muscular arteries.21
Cardiovascular manifestations can be prominent in the
acute phase of Kawasaki disease and are the leading cause
of long-term morbidity and mortality in these patients.
During this acute phase, the pericardium, myocardium,
endocardium, valves, and coronary arteries may all be
involved.
1861
Fig. 76.5: Left main coronary artery (LMCA; white arrow) and left
anterior descending artery (LAD; red arrow) ectasia in a patient
with Kawasaki disease without aneurysm.
1862
Table 76.1: Echocardiographic Views of Coronary Arteries in Patients with Kawasaki Disease
Left main coronary artery: Precordial short-axis at level of aortic valve; precordial long-axis of left ventricle (superior tangential);
subcostal left ventricular long-axis
Left anterior descending coronary artery: Precordial short-axis at level of aortic valve; precordial superior tangential long-axis of
left ventricle; precordial short-axis of left ventricle
Left circumflex: Precordial short-axis at level of aortic valve; apical four-chamber
Right coronary artery, proximal segment: Precordial short-axis at level of aortic valve; precordial long-axis (inferior tangential) of
left ventricle; subcostal coronal projection of right ventricular outflow tract; subcostal short-axis at level of atrioventricular groove
Right coronary artery, middle segment: Precordial long-axis of left ventricle (inferior tangential); apical four-chamber; subcostal
left ventricular long axis; subcostal short-axis at level of atrioventricular groove
Right coronary artery, distal segment: Apical four-chamber (inferior); subcostal atrial long-axis (inferior)
Posterior descending coronary artery: Apical four-chamber (inferior); subcostal atrial long-axis (inferior); precordial long-axis
(inferior tangential) imaging posterior interventricular groove
Fig. 76.6: Left main coronary artery (LMCA; white arrow) and left
anterior descending artery (LAD; yellow arrow) ectasia and early
aneurysm near the bifurcation (red arrow) in a patient with Kawasaki disease.
The most commonly used view is the parasternal shortaxis at the level of the aortic root. However, a compre
hensive study of both the left and right coronary arteries
and their branches should be performed from parasternal,
four-chamber, and subcostal views to ascertain full
evaluation of the coronary system (Table 76.1).
The coronary arteries should be measured by 2D
echocardiography from inner edge to inner edge excluding
points of branching. Particular attention should be paid
to describe coronary abnormalities, including ectasia,
aneurysm, or intraluminal thrombi. When a coronary
artery is larger than normal (dilated) without a segmental
aneurysm, the vessel is considered ectatic. The use of z
scores are available for the LMCA, proximal LAD, and
proximal RCA. Aneurysms are classified as saccular if
REFERENCES
1. Van Hare GF, Ben-Shachar G, Liebman J, et al. Infective
endocarditis in infants and children during the past
10 years: a decade of change. Am Heart J. 1984;107(6):
123540.
2. Li JS, Sexton DJ, Mick N, et al. Proposed modifications to
the Duke criteria for the diagnosis of infective endocarditis.
Clin Infect Dis. 2000;30(4):6338.
3. OBrien JT, Geiser EA. Infective endocarditis and echo
cardiography. Am Heart J. 1984;108(2):38694.
4. Gilbert BW, Haney RS, Crawford F, et al. Two-dimensional
echocardiographic assessment of vegetative endocarditis.
Circulation. 1977;55(2):34653.
1863
1864
SECTION 7
Miscellaneous and Other
Noninvasive Techniques
Chapters
Chapter 77
Chapter 78
Chapter 79
Chapter 80
Chapter 81
1867
CHAPTER 77
Echocardiography in
Systemic Diseases
Mahdi Veillet-Chowdhury, Smadar Kort
Snapshot
Rheumatoid Arthris
Hypereosinophilic Syndrome
Systemic Sclerosis
Renal Disease
Amyloidosis
INTRODUCTION
The presence of cardiac involvement in patients who suffer
various systemic diseases is relatively common and is often
associated with worse prognosis; therefore, early diagnosis
is critical. Echocardiography is a simple, noninvasive
imaging modality that is valuable in the evaluation of
patients with suspected cardiac manifestations of certain
disease processes, from autoimmune syndromes to
inflammatory conditions to various infections. In this
chapter, we will discuss the role of echocardiography in
the assessment of these patients and illustrate how newer
features of echocardiography such as tissue Doppler
imaging, three-dimensional (3D) echocardiography, and
speckle-tracking can be used to better assess specific
pathologies.
Carcinoid
Chagas Disease
Sarcoidosis
Thyroid Disorders
Nutrional Deficiency
1868
RHEUMATOID ARTHRITIS
Rheumatoid arthritis (RA) is a systemic autoimmune
disease of unknown etiology that can affect several organs,
including the heart. Cardiac manifestations include
coronary artery disease, conduction disease, valvular heart
disease, pericardial disease, and myocardial disease.12
Both systolic and diastolic LV dysfunction can occur
due to a combination of accelerated cardiovascular disease,
chronic inflammation, and use of cardiotoxic medications
causing nodules and fibrosis in the myocardium.13 Since
the myocardial performance index (MPI) is independent of
heart rate, preload, and afterload, it has been used to assess
global LV dysfunction in RA patients. A study involving
40 patients with active RA revealed that the MPI of the LV
was significantly higher than those of control patients,
indicative of abnormal global LV function, while the right
ventricular (RV) MPI was preserved.14 In combination with
HYPEREOSINOPHILIC SYNDROME
Hypereosinophilic syndrome (HES), or Loefflers syndrome,
is characterized by a persistently elevated eosinophil
count (1.5 109/L) for at least 6 months as well as
eosinophil-related end organ damage with no identifiable
cause. HES tends to occur more commonly in males
between the third and sixth decades of life. Cardiac
involvement has been reported in >50% of patients and
suggests a poor prognosis.24 Cardiac damage classically
occurs in two stages. In the first stage, acute myonecrosis
occurs due to eosinophilic infiltration of the myocardium
and subsequent release of toxic proteins. The later
stage is described by the formation of mural thrombi,
endomyocardial fibrosis, valvular disease, and restrictive
cardiomyopathy.25,26
1869
SYSTEMIC SCLEROSIS
Systemic sclerosis (SSc) is a connective tissue disorder
characterized by vascular lesions and widespread fibrosis
of the skin and other organs, frequently the heart, with
one study showing the prevalence of cardiac involvement
to be as high as 32% in patients with diffuse SSc.29 A
range of cardiac manifestations can be seen, including
conduction system disease, coronary artery disease,
pericardial involvement, RV dysfunction due to pulmonary
hypertension, and both LV systolic and diastolic
dysfunction. As studies have shown that patients with
cardiac involvement confer a poor prognosis, screening
for subclinical disease with echocardiography is vital.30
1870
Fig. 77.3: A 74-year-old gentleman with hypereosinophilic syndrome (HES). A two-dimensional (2D) apical four-chamber view
demonstrating thickening and infiltration of the right ventricle
(RV; arrow). (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).
Figs 77.4A to D: (A) A 67-year-old gentleman with asthma and hypereosinophilic syndrome (HES) with an eosinophil count of
5.6 109/L. A two-dimensional (2D) parasternal long-axis view demonstrating thickening and infiltration of the left ventricle (LV); (B) A
parasternal short-axis view demonstrating thickening and infiltration of the LV (arrow); (C) An apical four-chamber view demonstrating
thickening and infiltration of the LV (arrow); (D) An apical two-chamber view demonstrating thickening and infiltration of the LV. (LA: Left
atrium; LV: Left ventricle; RV: Right ventricle).
RENAL DISEASE
Cardiovascular mortality is significantly increased in
patients with chronic kidney disease (CKD), leading to
earlier atherosclerosis, valvular and pericardial disease,
arrhythmias, and heart failure. Use of echocardiography
plays a crucial role in the evaluation of these patients as
the structural and functional abnormalities can alter the
management and prognosis of this patient population.
Over time, a combination of CKD and other medical
1871
1872
Figs 77.5A and B: (A) A 59-year-old woman with endstage renal disease (ESRD). A transesophageal echocardiogram (TEE) demonstrating a large echodensity (arrows) on the atrial surface of the posterior mitral valve (MV) annulus and leaflet consistent with mobile
mitral annular calcification (MAC); (B) A TEE zoomed in view demonstrating a large echodensity (arrows) on the atrial surface of the
posterior MV annulus and leaflet consistent with mobile mitral annular calcification (MAC). (LA: Left atrium; LV: Left ventricle; RA: Right
atrium; RV: Right ventricle).
AMYLOIDOSIS
Amyloidosis is a systemic disorder that is caused by
extracellular deposits of insoluble aggregated proteins
with a -pleated sheath configuration. The incidence
of light chain (AL) amyloidosis or primary amyloidosis
is more than 10 per million person-years in the US
population and has a predilection for men in the sixth
decade of life.69 Amyloid can affect numerous organ
systems but infiltration of the heart confers an extremely
poor prognosis, as amyloid patients with congestive heart
failure (CHF) have a median survival of about 6 months.70
In cardiac amyloidosis, amyloid deposits infiltrate the
myocardium with progression to myocyte necrosis and
local interstitial fibrosis.
As the heart is thickened, diastolic dysfunction progresses, eventually leading to restrictive cardiomyopathy.71
Due to the reduced compliance of the LV, the chamber
diameters remain normal, but the free wall and septum
thicken.72 While amyloid deposits are rare in the epicardial
1873
Fig. 77.6: An 89-year-old woman with systemic amyloidosis. Threedimensional (3D) apical four-chamber view showing right ventricle
(RV) infiltration, can be used to differentiate thickening of the free
wall from a moderator band (asterisk). (LA: Left atrium; LV: Left
ventricle; RA: Right atrium; RV: Right ventricle).
1874
CARCINOID
Carcinoid tumors are rare neuroendocrine malignancies
derived primarily from enterochromaffin cells, with the
primary site usually being in the gastrointestinal tract. The
usual presentation occurs in the fifth to seventh decade of
life with the classic symptom triad of flushing, secretory
diarrhea, and bronchospasm.84 Carcinoid heart disease
has been reported to be present in up to 5060% of patients
1875
CHAGAS DISEASE
Chagas disease is a tropical disease endemic in Mexico,
Central, and South America caused by the parasite
Trypanosoma cruzi. There is usually an acute or early
phase and a chronic or late phase. Although infrequent,
fulminant myocarditis can occur in 15% of patients in the
acute phase.98 More commonly, about 30% of seropositive
individuals develop cardiomyopathy several years after the
acute phase.99 This usually occurs through a combination
of cardiac dysautonomia, microvascular disease, parasite,
and immune-mediated myocardial injury. A recent
systematic review demonstrated that positive serology for
Chagas disease is associated with a higher risk of death in
patients with heart failure.100
Echocardiography can provide valuable information
regarding the extent of myocardial damage and, therefore,
help determine the prognosis of patients with Chagas heart
disease. Apart from acute myocarditis, the early phase
of Chagas disease can lead to the rapid development of
pericardial effusions, with one study noting the presence
of pericardial effusions in 42% of subjects.101 Most of the
structural heart changes take place slowly over years
after the initial infection goes unnoticed (Movie clip 77.5;
Figs 77.11A to F). Studies have shown that > 50% of
patients with Chagas disease develop apical aneurysms,
and become at higher risk of forming a mural thrombus.102
Both TTE and TEE were shown to recognize potential
cardiac source of emboli with high accuracy.103
More advanced cardiac disease include global cardiac
dilatation and diffuse hypokinesis; however, early in the
disease process, segmental wall motion abnormalities
can also be identified on 2D echocardiograms, with the
apical and posteroinferior walls being the most commonly
1876
Figs 77.9A to D: A 53-year-old gentleman with metastatic carcinoid disease to bones and liver. A two-dimensional (2D) echocardiography
showing a thickened, retracted, and noncoapting tricuspid valve (arrow); (B) A color flow Doppler echocardiography showing severe
tricuspid regurgitation with a large regurgitant jet; (C) A continuous wave (CW) Doppler echocardiography showing an increased
E-velocity across the tricuspid valve (TV), consistent with severe tricuspid stenosis (curved arrow). However, the mean pressure gradient
of 5 mm Hg across the valve excludes the presence of significant stenosis as the etiology; (D) A three-dimensional (3D) short-axis view
demonstrating a thickened, fixed, noncoapting tricuspid valve (arrow). (RA: Right atrium; RV: Right ventricle).
SARCOIDOSIS
Sarcoidosis is a multisystem disorder that is characterized
by the presence of noncaseating granulomas that can
affect numerous organs in the body. The etiology remains
unknown, although environmental, occupational, infectious, and genetic causes have all been proposed. While
1877
Figs 77.10A and B: (A) A 53-year-old gentleman with carcinoid heart disease. A two-dimensional (2D) short-axis view showing a thickened and fixed pulmonic valve (PV; arrow); (B) A continuous wave (CW) Doppler echocardiography showing mild pulmonic stenosis with
a mean transvalvular pressure gradient of 20 mm Hg (curved arrow).
Figs 77.11A to D
1878
Figs 77.11A to F: A 28-year-old gentleman emigrated from El Salvador and presented with Chagas heart disease. A two-dimensional
(2D) parasternal long-axis view showing a dilated left ventricle (LV; asterisk); (B) A 2D echocardiogram short-axis view showing a dilated
LV (asterisk); (C) A 2D apical four-chamber view showing a dilated LV with a thinned apex (arrow); (D) A zoomed-in 2D apical fourchamber view showing mitral regurgitation (MR); (E) A 2D apical four-chamber view showing a dilated LV with a thinned apex (arrow);
(F) A 2D apical four-chamber view showing a dilated LV with a thinned apex (arrow) (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).
THYROID DISORDERS
Hyperthyroidism is characterized by elevated peripheral
free thyroid hormone levels combined with decreased
thyroid stimulating hormone (TSH) levels. Thyrotoxicosis
can lead to significant changes in the cardiac structure
and function, causing hypertension, heart failure, and
arrhythmias. Echocardiography can play an important
role in the evaluation of patients with thyroid disorders,
as even subclinical hyperthyroidism can lead to cardiac
abnormalities. Elevated thyroid hormones leads to a high
cardiac output by up to 50300% as well as increased
preload, leading to an increase in LV mass and LA
size.121124 Due to the increased cardiac contractility and
heart rate, shortened interventricular conduction time
and pre-ejection period have been demonstrated.123 SRI
has shown enhancement of systolic stain rate in patients
with subclinical hyperthyroidism due to the greater
early systolic phase deformation.125 However, stress
echocardiography has demonstrated a blunted increase
in LV ejection fraction and cardiac output.126 In regards
to RV function, TDI has demonstrated enhanced systolic
function in subjects with overt hyperthyroidism.127
Mild diastolic dysfunction has also been demonstrated
by Doppler echocardiography, with reduced peak E-wave
velocity and a significantly higher peak A-wave velocity.125
TDI reveals impairment of the mitral annular velocity and
1879
Fig. 77.13: A 74-year-old woman with sarcoidosis. A twodimensional (2D) apical four-chamber view demonstrates
right ventricular (RV) hypertrophy, mildly dilated RV, and right
atrium (RA). LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle.
1880
Figs 77.14A and B: (A) A 59-year-old gentleman with hypothyroidism. A two-dimensional (2D) parasternal long-axis view demonstrating a dilated left ventricle (LV); (B) An apical four-chamber view demonstrating dilated atria and LV. (LA: Left atrium; LV: Left ventricle;
RA: Right atrium; RV: Right ventricle).
Figs 77.15A and B: (A) A 46-year-old gentleman with a history of alcohol abuse and thiamine deficiency. A two-dimensional (2D) apical
two-chamber view showing a dilated left ventricle (LV) with reduced systolic function; (B) A 2D apical two-chamber view showing small
LV diameter and significantly improved systolic function after thiamine replacement. (LA: Left atrium; LV: Left ventricle).
NUTRITIONAL DEFICIENCY
Nutritional deficiencies in patients can adversely affect
myocardial performance and increase cardiovascular
morbidity and mortality, particularly thiamine deficiency
due to either dietary factors or alcohol abuse (or sometimes
a combination of both). Thiamine, or Vitamin B1, is a watersoluble B complex vitamin, and its deficiency has been
shown to cause heart disease, most notably beriberi heart
disease. Beriberi is characterized by heart failure with
biventricular dysfunction with significant hemodynamic
abnormalities, most particularly RV failure and elevated
LV end-diastolic pressures.140 Echocardiographic features
include biventricular enlargement and decreased systolic
function of both chambers (Figs 77.15A and B).
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35. Meune C, Allanore Y, Pascal O, et al. Myocardial contractility is early affected in systemic sclerosis: a tissue Doppler echocardiography study. Eur J Echocardiogr. 2005;
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49. Tripepi G, Benedetto FA, Mallamaci F, et al. Left atrial
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50. Sharma R, Pellerin D, Gaze DC, et al. Mitral peak Doppler
E-wave to peak mitral annulus velocity ratio is an accurate
estimate of left ventricular filling pressure and predicts
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hypothyroidism. Int J Cardiovasc Imaging. 2006;22(2):
17786.
134. Kosar F, Sahin I, Turan N, et al. Evaluation of right and
left ventricular function using pulsed-wave tissue Doppler
echocardiography in patients with subclinical hypothyroidism. J Endocrinol Invest. 2005;28(8):70410.
135. Doin FL, Borges Mda R, Campos O, et al. Effect of central
hypothyroidism on Doppler-derived myocardial performance index. J Am Soc Echocardiogr. 2004;17(6):6229.
136. Biondi B, Klein I. Hypothyroidism as a risk factor for
cardiovascular disease. Endocrine. 2004;24(1):113.
1885
CHAPTER 78
Echocardiography in Women
Jennifer Kiessling, Navin C Nanda, Tugba Kemaloglu z, Aylin Sungur, Kunal Bhagatwala, Nidhi M Karia
Snapshot
INTRODUCTION
In terms of cardiac disease, it is often thought that men
are more affected, while women are somehow protected.
This has proven to be untrue. This chapter will illustrate
the use of the echocardiogram in women. Furthermore,
it will highlight some differences in the standardization
of echocardiographic measurements as well as some
technical challenges in women. Echocardiography is often
the primary diagnostic tool to diagnose patients with
structural heart disease. Structural heart diseases such
as atrial septal defects, mitral valve prolapse (MVP), and
pulmonary arterial hypertension are more commonly seen
in women. Also, the utilization of stress echocardiography
in women to diagnose underlying coronary artery disease
(CAD) is discussed and compared to other current
imaging modalities. The usage of the echocardiogram in
pregnant women is discussed in detail. Finally, the role of
fetal echocardiography is highly useful to diagnose and
manage fetal cardiac anomalies.
Takotsubo Cardiomyopathy
1887
Table 78.1: Reference Limits and Partition Values of Left Ventricular Mass and Geometry
Women
Men
Reference
Range
Mildly
Abnormal
Moderately
Abnormal
Severely
Abnormal
Reference
Range
Mildly
Abnormal
Moderately
Abnormal
Severely
Abnormal
67162
163186
187210
211
88224
225258
259292
293
4395
96108
109121
122
49115
116131
132148
149
Linear Method
LV mass, g
2
LV mass/BSA, g/m
LV mass/height, g/m
4199
100115
116128
129
52126
127144
145162
163
LV mass/height2,7, g/m2,7
1844
4551
5258
59
2048
4955
5663
64
0.220.42
0.430.47
0.480.52
0.53
0.240.42
0.430.46
0.470.51
0.52
Septal thickness, cm
0.60.9
1.01.2
1.31.5
1.6
0.61.0
1.11.3
1.41.6
1.7
1.01.2
1.31.5
1.6
0.61.0
1.11.3
1.41.6
1.7
66150
151171
172182
> 193
96200
201227
228254
> 255
4488
89100
101112
113
50102
103116
117130
131
2D Method
LV mass, g
2
LV mass/BSA, g/m
Source: Reproduced from Roberto M. Lang, Michelle Bierig, Richard B. Devereux, et al. Recommendations for Chamber Quantification:
A Report from the American Society of Echocardiographys Guidelines and Standards Committee and the Chamber Quantification
Writing Group, Developed in Conjunction with the European Association of Echocardiography, a Branch of the European Society of
Cardiology. J Am Soc Echocardiogr. 2005;18:144063.
(BSA: Body surface area; LV: Left ventricular; 2D: 2-dimensional).
Bold italic values: Recommended and best validated.
Figs 78.1A and B: Breast implant. Two-dimensional transthoracic echocardiography. (A) Arrows point to images produced by a saline
breast implant; (B) Apical four-chamber view. Arrow points to a breast implant and the arrowhead to a pacing wire in the region of the
tricuspid valve, which shows fatty infiltration (F). A prominent moderator band (M) is seen in the right ventricle. (LA: Left atrium; LV: Left
ventricle; RA: Right atrium). (Movie clips 78.1A and B).
1888
Figs 78.2A and B: Mitral valve prolapse in a 42-year-old patient. (A) Two-dimensional transthoracic echocardiography. Arrowhead
points to prolapse of both mitral leaflets. (B) M-mode study was useful in demonstrating prolapse in mid to late systole (arrow).
(AO: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral valve; RV: Right ventricle; SVC: Superior vena cava). (Movie clip 78.2).
1889
Figs 78.3A and B: Ruptured chordae tendinae and mitral valve prolapse in a 71-year-old female. Live/real time three-dimensional
transesophageal echocardiography. (A) The arrowhead shows a ruptured chord of a severely prolapsing A2 segment of the anterior
mitral valve leaflet (AML); (B) Color Doppler imaging. Numbers 1 and 2 point to two jets of severe MR. The arrowhead points to the ruptured chord. (LA: Left atrium; MV: Mitral valve; MR: Mitral regurgitation; PML: Posterior mitral valve leaflet). (Movie clips 78.3A and B).
Source: Reproduced with permission from Nanda et al. Comparison of real time two-dimensional with live/real time three-dimensional
transesophageal echocardiography in the evaluation of mitral valve prolapse and chordae rupture. Echocardiography. 2008;25:11317.
1890
Figs 78.4A to F: Rheumatic mitral stenosis. Two-dimensional transthoracic echocardiography in a 45-year-old female with
a history of rheumatic fever several years ago. (A) Arrow points to thickened mitral valve leaflets with a typical hockey-stick
appearance visualized in the parasternal long axis view; (B) Short-axis view of the mitral valve (MV) at the leaflet tips. MV area
measured 1.10 cm2 suggestive of fairly severe stenosis; (C) Color Doppler examination shows a turbulent jet (arrowhead) in the
left ventricle (LV) originating from the MV. Note the prominent flow acceleration (arrow); (D) Color Doppler guided continuouswave Doppler show peak and mean gradients of 34 and 19 mm Hg, respectively; (E) Pressure half time assessment also
showed severe MV stenosis with a valve area of 0.87 cm2; (F) Live/real time three-dimensional study confirmed the presence of
severe stenosis with no calcification of commissures. There was only mild mitral regurgitation. The patient is on the wait list for
percutaneous mitral valvuloplasty (Movie clips 78.4A, C, and F). (AO: Aorta; LA: Left atrium; LV: Left ventricle; MV: Mitral valve;
RV: Right ventricle).
1891
CAD on coronary angiography, compared to men. Interestingly, women often times have a more adverse outcome
despite having lower angiographic disease burden.
1892
Figs 78.6A and B: Female patient with rheumatic involvement of mitral and tricuspid valves. Transthoracic three-dimensional echocardiography. (A) Apical four-chamber view showing thickened mitral (MV) and tricuspid (TV) valves. (B) Cropping of the three-dimensional
data set and en face viewing shows a very small mitral orifice in diastole indicative of severe stenosis. Note absence of calcification in
the commissures. In comparison, the TV shows a much larger opening consistent with absence of significant stenosis. The movie clip
shows fairly preserved motion of anterior (A) and posterior (P) leaflets but marked restriction of the septal (S) leaflet of the tricuspid
valve. The septal leaflet is identified by its close proximity to the ventricular septum. The anterior and posterior leaflets are recognized
by their anterior and posterior locations in relation to the left ventricular outflow (LVO) tract. The three-dimensional technique is considered the gold standard for assessing the mitral orifice area because, unlike two-dimensional echocardiography, the cropping plane can
be positioned exactly parallel to the flow limiting orifice tip. Also, three-dimensional echocardiography easily assesses all three leaflets
of the TV en face which is difficult with two-dimensional imaging (Movie clip 78.6). (LA: Left atrium; LV: Left ventricle; RA: Right atrium;
RV: Right ventricle).
A
Figs 78.7A and B
1893
Figs 78.7A to D: Mitral stenosis and regurgitation in a female patient. Transthoracic three-dimensional echocardiography. (A) Apical
four-chamber view shows restricted mobility of both mitral valve (MV) leaflets which are thickened; (B) Cropping of the three-dimensional
data set was performed to view the mitral orifice (arrow) at its tip. Planimetry was consistent with significant stenosis. Note presence of
calcification in the body of the posterior leaflet but the anterior leaflet and both commissures are free of calcium; (C) Mosaic color signals
in the left atrium with a prominent flow acceleration point to severe mitral regurgitation; (D) Meticulous cropping of the three-dimensional
data set was done to view en face the vena contracta of the mitral regurgitation jet. This was performed practically at the level of the
mitral leaflets between the flow acceleration and the regurgitant jet. It measured more than 0.6 cm2 indicative of severe regurgitation
(Movie clip 78.7). Other abbreviations as in previous figures.
Figs 78.8A and B: (A) Rheumatic mitral stenosis and regurgitation in a female patient. Transesophageal two-dimensional echocardiography. Four-chamber view shows thickening of both mitral leaflets with restricted motion. Note thickening of chordal apparatus also;
(B) Color Doppler examination demonstrates significant mitral regurgitation (MR) (Movie clips 78.8A and B). Other abbreviations as in
previous figures.
shifting energy substrates toward myocardial and peripheral glucose metabolism. Therefore, the traditional
stress testing which is based on demand ischemia may fail
to detect obstructive CAD in subsets of women without a
significant coronary stenosis.13
1894
Figs 78.9A and B: Rheumatic mitral stenosis and regurgitation in a female patient. Transesophageal three-dimensional echocardiography. (A) En face view of the mitral valve (MV) shows mild narrowing of the thickened MV consistent with mild to moderate
stenosis; (B) Systolic view shows noncoaptation of the mitral leaflets indicative of significant mitral regurgitation. (AV: Aortic valve).
(Movie clip 78.9).
Figs 78.10A and B: Rheumatic mitral stenosis in another female patient. Transesophageal three-dimensional echocardiography.
(A) The left atrium is viewed from the top and shows a large clot attached to the supero-posterior wall. The mitral valve (MV) is heavily
calcified; (B) Three-dimensional two-chamber view demonstrates clots (arrowheads) in the body and appendage of the left atrium
(Movie clips 78.10A and B). Abbreviations as in previous figures.
1895
1896
B
obstructive CAD. More recently, live/real time threedimensional (3D) echocardiography is increasingly used
together with contrast echocardiography in an attempt to
further enhance the accuracy of stress echocardiography
in assessing CAD. With 3D echocardiography, it is
possible to capture the whole LV in the pyramidal shaped
3D data set which can then be cropped in a systematic
and sequential manner to examine all ventricular walls
and segments for stress induced motion abnormalities.
This obviates the limitations of the 2D technique which
produces only thin slice-like sections of the LV at any
given time precluding comprehensive assessment of
all segments. Apical foreshortening, common with 2D
echocardiography, is avoided or reduced with the 3D
approach which also has been shown to have much less
intra- and interobserver variability in the assessment of
left ventricular function. Another advantage is the 3D
data can be stored in the equipment or offline and can
Figs 78.13A to F
1897
1898
Figs 78.13G to L
1899
Figs 78.13A to M: Coronary stenosis in a 61-year-old female. Transesophageal echocardiographic examination. (A) Top arrow points
to dilatation of the proximal left anterior descending coronary artery
(LAD), whereas the bottom arrow demonstrates turbulent flow in
mid-LAD. Pulsed Doppler interrogation of this area revealed a high
diastolic velocity of 1.3 m/s (arrowhead in the inset), consistent
with significant stenosis; (B) Top arrow points to turbulent flow in
mid-LAD, whereas the bottom arrow shows turbulent flow in the
more distal portion of LAD; (C) Arrow demonstrates narrowing in
the first diagonal branch (arrowhead) of LAD. Continuous-wave
Doppler interrogation reveals a very high diastolic velocity of 3.0
m/s (arrowhead in the inset) indicative of very severe stenosis; (D)
Arrows show large segments of proximal, mid and distal segments
of LAD visualized in this view; (E) Arrow demonstrates the presence
of turbulent flow in the proximal right coronary artery; (F) Pulsed
Doppler interrogation (arrowhead) of right coronary artery (RCA)
M
demonstrates a high velocity of 1.3 m/s (arrowhead in the inset)
indicative of significant stenosis; (G) Arrow points to the presence of turbulent flow in the left circumflex coronary artery; (H) Arrow points
to the origin of the left circumflex coronary, which appears normal. However, reversed flow (blue) with turbulent flow signals (arrowhead)
is noted in the mid and distal portions of the circumflex vessel, consistent with filling from collaterals and significant stenosis. Doppler
interrogation of this area shows a high diastolic velocity of 1.0 m/s (arrowheads in the inset); (I) Arrowhead demonstrates turbulent flow
signals in the intramyocardial coronary arteries consistent with stenosis; (J) The interventricular vein (V) is imaged next to the dilated
LAD (arrow). Note that the flow in the interventricular vein is in the opposite direction of LAD flow; (K) Coronary angiogram. The top
arrowhead demonstrates 90% stenosis at the origin of the diagonal branch. The proximal LAD is dilated, and beyond the dilatation,
the mid-LAD shows 50% stenosis (just beyond the bottom arrowhead). Note multiple areas of significant stenosis in the more distal
segments of LAD; (L) Arrow points to total occlusion of proximal circumflex coronary artery. Retrograde filling of more distal portions of
the circumflex vessel from collaterals was noted; (M) Coronary angiogram. The arrow points to significant stenosis in the proximal right
coronary artery. Note significant stenosis in the mid and distal portions. (AO: Aorta; LA: Left atrium; LM: Left main coronary artery; LV:
Left ventricle; MV: Mitral valve; PA: Pulmonary artery; RVO: Right ventricular outflow tract). Reproduced with permission from Nanda
et al. Transesophageal echocardiographic diagnosis of coronary stenosis in a stroke patient. Echocardiography. 1999;16:58992.
In Movie clip 78.13, 1, Left anterior descending coronary artery; 2, Diagonal branch; 3, Proximal right coronary artery; 4, circumflex
artery; 5, Intramyocardial coronary arteries (cannot be visualized by angiogram). V, coronary vein. Upper arrow shows proximal LAD.
Lower arrow shows distal LAD. Arrowhead points to post-stenotic dilatation of the diagonal branch.
TAKOTSUBO CARDIOMYOPATHY
Takotsubo cardiomyopathy is a very common clinical
entity which occurs far more often in women than in
1900
Vector velocity imaging (VVI) has been used to demonstrate that in patients with Takotsubo cardiomyopathy,
there is both LV systolic and diastolic longitudinal dysfunction, not just systolic radial dysfunction.11 Traditional
2D echocardiography is typically used to assess radial dysfunction. VVI uses a tracking algorithm which incorporates both velocities of set points (i.e. mitral annulus and
tissue-cavity border) as well as speckle tracking. The result
is segmental quantitative velocity, strain, and strain rate.
VVI has demonstrated that there are definitive reductions
in both longitudinal systolic and diastolic dysfunction in
Takotsubo cardiomyopathy.11 It has also been shown that
there is improvement in the longitudinal function with
time. Furthermore, there has been evidence to suggest that
the left atrium is also affected in Takotsubo cardiomyopathy.11 Specifically, the left atriums systolic strain and strain
rate and the diastolic velocity and strain rate are reduced.
However, there is some data to suggest that there is improvement in the involved walls of the left atrium.11
1901
Fig. 78.15: Secundum atrial septal defect in a 37-year-old female. Two-dimensional transesophageal echocardiography. Arrow points
to flow signals moving from the left atrium into the right atrium through a secundum defect. Abbreviations as in previous figure. Movie
clip 78.15 is from another female patient demonstrating en face visualization of a secundum atrial septal defect (upper arrow) using
live/real three-dimensional tranesophageal echocardiography. Note a large rim in relation to the aorta (AO), tricuspid valve (lower left
arrow), and mitral valve (MV). Lower right arrow points to the pulmonary valve. (PA: Pulmonary artery). Other abbreviations as in previous figure.
Pulmonary Hypertension
Pulmonary arterial hypertension is typically defined as
a measured mean pulmonary artery pressure (mPAP)
1902
Figs 78.16A to D: Severe systemic level pulmonary artery pressure in a 37-year-old female with primary pulmonary hypertension.
Two-dimensional transthoracic echocardiography. Apical views. (A) Note the marked enlargement of both the right ventricle (RV) and
the right atrium (RA). The atrial septum bulges prominently into the left atrium (LA). Movie clip 78.8A shows diastolic bulging of the ventricular septum into the left ventricle (LV) indicative of right-sided volume overload; (B) Color Doppler examination shows the presence
of severe tricuspid regurgitation (TR); (C) Continuous-wave Doppler examination reveals a very high pulmonary artery systolic pressure
of 118 mm Hg (arrow); (D) Bubble study shows delayed appearance (after 4 beats) of the micro bubbles (arrowhead) in the left heart
consistent with intrapulmonary shunting. This is related to dilatation of the pulmonary arterioles. (LV: Left ventricle; TV: Tricuspid valve).
(Movie clips 78.16A to C).
ECHOCARDIOGRAPHY IN PREGNANCY,
PERIPARTUM CARDIOMYOPATHY,
FETAL ECHOCARDIOGRAPHY
Many physiological changes develop in many organ
systems during the course of pregnancy. Cardiac output
increases by 30% to 40% during the first trimester of
1903
Figs 78.17A and B: Severe pulmonary hypertension with right-sided involvement. Two-dimensional transthoracic echocardiography.
(A and B) The tricuspid valve (TV) annulus is dilated with systolic noncoaptation (arrow) of the leaflets leading to severe tricuspid regurgitation (arrowhead). The pulmonary annulus is also dilated resulting in severe pulmonic valve regurgitation (PR). (CS: Coronary sinus;
DA: Descending aorta; PV: Pulmonary valve; RA: Right atrium; RV: Right ventricle). (Movie clips 78.17A and B).
1904
pregnancy. These changes can be seen on Doppler echocardiography with increased blood flow velocities and
more prominent color Doppler flow signals in the cardiac
cavities. Increased color Doppler flow signals in the region
of the atrial septum may mimic an atrial septal defect
especially in the presence of septal dropouts common in
the apical four-chamber view. In this instance, continuity
of color flow signals between the two atria in the region
of the septal dropout may result in an erroneous diagnosis
of a secundum atrial septal defect. At 8 to 11 weeks,
an average cardiac output of 6.7 L/min will increase to
8.7 L/min at 36 to 39 weeks. This increase is mostly due to
an increase in stroke volume, but also a more rapid
heart rate. Also, there is a reduction in systemic vascular
resistance. It is these changes which characterize the
hyperdynamic circulation seen in pregnancy. On the
echocardiogram, hyperkinetic ventricular wall motion is
evident during this period of pregnancy.
Peripartum Cardiomyopathy
Figs 78.19A to D
1905
Figs 78.19A to G: Peripartum cardiomyopathy. Live/real time three-dimensional transthoracic echocardiography in a 29-year-old
female. (A) Arrow points to a large clot in the left ventricular (LV) apex. Both ventricles showed poor function; (B to D) A transverse plane
(TP) section through the clot (B) shows a large area of echolucency (arrow) consistent with clot lysis (C and D). This indicated that the
clot had practically completely dissolved and only a thin rim remained; (E) Schematic showing coagulation cascade and thrombus lysis.
Plasminogen-activating factor (PAF) is secreted by endothelialized mesenchymal cells lining the microscopic crypts which develops
within the thrombus. PAF activates plasminogen to plasmin which digests fibrin leading to thrombus lysis. (F and G) A TP section taken
at the attachment point of the clot shows it to be highly echogenic consistent with collagen. This patient had been on anticoagulant
therapy for a long time but on the two-dimensional study the clot did not appear to regress. However, the three-dimensional technique
was useful in assuring us the effectiveness of anticoagulant therapy with almost complete clot resolution. The remainder of the clot
regressed completely with no clinical or laboratory evidence of embolization (Movie clips 78.19). (vWF: von Willebrand factor; TF: Tissue
factor; WBC: White blood cell; RBC: Red blood cell; CF: Clotting factors). Other abbreviations as in previous figures.
1906
Fetal Echocardiography
Figs 78.20A to F
1907
Figs 78.21A to D
1908
Figs 78.21E to J
1909
Figs 78.21A to N: Complete atrioventricular septal defect in 36-week-old fetus. Live/real time three-dimensional echocardiographic
study. (A to D) Four-chamber views cropped to show the common atrioventricular valve (V) and the defect (asterisks). Arrowhead in C
points to the atrial septum. (E to F) The pyramidal section has been cropped from the top and rotated toward the examiner to display all
five leaflets of V: posterior (P), left lateral (L1), left anterior (A1), right anterior (A2), and right lateral (L2). Small portions of the ventricular
septum (S) and atrial septum (AS) have been retained to show their relationship to V. V is open in E and closed in F. (G) Arrowhead
demonstrates multiple chordal attachments of V to S; (H to J) En face viewing of the defect (asterisk) from above (H), from the inferior
aspect (I) and from the right side (J); (K to M) Five-chamber view shows the aorta (AO) arising from LV. In M, the pyramidal section has
been cropped to show regurgitation (R) from the right-sided component of V. (N) Arrowhead shows the ductus arteriosus. (AV: Aortic
valve; S: Ventricular septum). (Movie clip 78.21).
Source: Reproduced with permission from Maulik et al. Live three-dimensional echocardiography of the human fetus. Echocardiography
2003;20:71521.
1910
Figs 78.23A to D: Systemic hypertension. The patient is a 37-year-old female with very severe hypertension. Two-dimensional transthoracic echocardiography. Parasternal long-axis (A), short-axis (B), and apical four-chamber (C) views. Note the marked concentric
hypertrophy of both the ventricular septum (VS) and the posterior wall (PW). The left atrial appendage (LAA) is clearly delineated and
is free of any clot. (D) M-mode study shows left ventricular hypertrophy. (AO: Aorta; LA: Left atrium; MV: Mitral valve; RA: Right atrium;
RV: Right ventricle). (Movie clips 78.23A to C).
1911
Fig. 78.25: Mitral valve vegetations in a 43-year-old female with infective endocarditis. Live/real time three-dimensional transesophageal echocardiography. The black arrow (horizontal arrowhead
in the video clip) points to a mitral valve vegetation involving the
A1 segment and commissure. Note the presence of central perforation. Two other vegetations are also seen involving A2 and A3
segments of the anterior mitral leaflet. (AO: Aorta; PV: Pulmonary
valve; TV: Tricuspid valve). (Movie clip 78.25).
Source: Reproduced with permission from Hansalia et al. The
value of live/real time three-dimensional transesophageal echocardiography in the assessment of valvular vegetations. Echocardiography. 2009;26:126473.
Figs 78.26A and B: Mitral valve vegetation in a 32-year-old female with infective endocarditis. Live/real time three-dimensional
transesophageal echocardiography. (A) The arrowhead points to a large mitral valve vegetation with a central perforation; (B) The
lower arrowhead points to an annular abscess and the upper arrowhead points to the mitral valve. (AO: Aorta; PV: Pulmonary valve).
(Movie clips 78.26A and 78.28B).
Source: Reproduced with permission from Hansalia et al. The value of live/real time three-dimensional transesophageal echocardiography in the assessment of valvular vegetations. Echocardiography. 2009;26:126473.
1912
Figs 78.27A and B: Right-sided heart failure resulting from severe tricuspid regurgitation due to infective endocarditis. Two-dimensional
transthoracic echocardiography. (A) Arrowheads denote vegetations on the tricuspid valve leaflets; (B) Color Doppler examination
shows severe tricuspid regurgitation (TR). (AO: Aorta; CS: Coronary sinus; LV: Left ventricle; RA: Right atrium; RV: Right ventricle).
(Movie clips 78.27A part 1, 78.27A part 2 and 78.27B).
Figs 78.28A and B: Systemic lupus erythematosus in a young female patient. Two-dimensional transthoracic echocardiogram. (A and B).
Parasternal long-axis view shows the presence of pericardial effusion (PE, A) which resolved during follow-up (B). There are no valvular
abnormalities. (AO: Aorta; CS: Coronary sinus; DA: Descending aorta; LA: Left atrium; LV: Left ventricle; PW: Posterior wall; RV: Right
ventricle; SVC: Superior vena cava; VS: Ventricular septum). (Movie clips 78.28A and B).
1913
Figs 78.29A and B: Metastatic left pleural effusion in a 53-year-old female with chronic myeloid leukemia. Two-dimensional transthoracic echocardiography. (A) In the parasternal long-axis view, the pleural effusion (PLE) is diagnosed by noting an echo free-space posteriorly extending beyond the descending thoracic aorta (DA); (B) PLE is readily diagnosed by placing the ultrasound transducer in the
posterior intercostal spaces with the patient sitting up. (LA: Left atrium; LB: Left back; LU: Lung; LV: Left ventricle; RV: Right ventricle).
(Movie clips 78.29A and B).
Figs 78.30A and B: Bicuspid aortic valve in a young female. (A and B) Both two-dimensional (A: Left panel, systole; right panel,
diastole) and three-dimensional (B) transthoracic echocardiography show the presence of a bicuspid aortic valve with horizontal cusp
orientation. (AO: Aorta; LA: Left atrium; PV: Pulmonary valve; RA: Right atrium, RV: Right ventricle). (Movie clips 78.30A and B).
1914
Figs 78.31A and B: Bicuspid aortic valve. Two-dimensional transesophageal echocardiogram showing a bicuspid aortic valve imaged
in long (A) and short (B) axis views. The arrow in A points to valve redundancy and prolapse into the left ventricular (LV) outflow tract.
In B, the cusps are vertically oriented and appear to be equal in size. (AO: Aorta; LA: Left atrium; RA: Right atrium; RV: Right ventricle;
TV: Tricuspid valve). (Movie clips 78.31A and B).
Figs 78.32A to D
1915
Fig. 78.33: Muscular outflow ventricular septal defects. Twodimensional transthoracic echocardiography. Short-axis view
at the level of the LV outflow tract demonstrates two adjacent
ventricular septal defects (1 and 2). Note the two defects are
not related to the tricuspid or pulmonary valve and hence are
not in the perimembranous or supracristal location. (AO: Aorta;
PV: Pulmonary valve; RA: Right atrium; RV: Right ventricle; TV:
Tricuspid valve). (Movie clip 78.33).
1916
Figs 78.34A to D: Levo (corrected) transposition of the great vessels with a ventricular septal defect and pulmonary artery banding in
a 25-year-old female. Parasternal long-axis views (A and B) show a large ventricular septal defect with flow signals moving from the
pulmonary ventricle (morphological LV) into the aorta (AO). The arrow points to a band in the pulmonary artery (PA) surgically placed
in an attempt to protect the patient from pulmonary hypertension. Two-dimensional (C) and three-dimensional (D) transthoracic shortaxis views show the aortic valve and the AO located directly anterior to pulmonary valve (PV) and PA indicative of transposition of the
great vessels. (CS: Coronary sinus; LV: Left ventricle; MV: Mitral valve; RA: Right atrium). (Movie clips 78.34A to D).
A
Figs 78.35A and B
1917
Figs 78.36A to D
1918
Figs 78.36A to F: Combined valvar and supravalvar aortic stenosis. Live/real time three-dimensional transthoracic echocardiography.
(A) Horizontal arrowhead points to supravalvar aortic stenosis produced by calcification at the sinotubular junction. The vertical arrowhead shows heavy mitral annular calcification; (B) Supravalvar stenotic orifice viewed in short axis (arrowhead); (C) Short-axis view at
the level of the aortic valve (AV) leaflets demonstrating mild valvar stenosis. Live/real time three-dimensional transthoracic echocardiography in a patient with calcification at the aortic sinotubular junction but no stenosis; (D) The arrowheads point to prominent calcifications
at the sinotubular junction viewed in long axis; (E) Short-axis view at the sinotubular junction shows a large orifice (arrowhead) that
measured 2.5 cm2; (F) Short-axis view at the level of the AV (left) and immediately above it (right). The AV orifice measured 1.7 cm2 by
planimetry, consistent with mild aortic stenosis. The arrowhead in the right panel points to sinotubular calcification protruding into the
aortic lumen imaged just beyond the AV leaflets. (AO: Ascending aorta; LA: Left atrium; LV: Left ventricle; PA: Pulmonary artery; RA:
Right atrium; RV: Right ventricle; RVO: Right ventricular outflow tract; TV: Tricuspid valve). (Movie clip 78.36).
Source: Reproduced with permission from Rajdev et al. Live/real time three-dimensional transthoracic echocardiographic assessment
of combined valvar and supravalvar aortic stenosis. Am J Geriatr Cardiol. 2006;15:18890.
A
Figs 78.37A and B
1919
Figs 78.37A to D: Paravalvular mitral regurgitation in a 69-year-old female. Paravalvular mitral regurgitation (arrowhead) is noted by
transthoracic (A) and transesophageal (B) echocardiography. (C and D) Represent live/real time three-dimensional images showing two
plugs (arrowheads) used to successfully close the leak percutaneously in the cardiac catheterization laboratory. Small arrows in D point
to intact sutures. (AV: Aortic valve; LA: Left atrium; LV: Left ventricle; MR: Mitral regurgitation; MVR: Mitral valve replacement; RA: Right
atrium; RV: Right ventricle). (Movie clips 78.37A to D).
CONCLUSION
Echocardiography proves to be a very useful diagnostic
tool for women, but it is not without its challenges. There
are structural heart diseases that occur more commonly in
women, and it is important to remember that women can
often have atypical presentations, which sometimes delays
the accurate diagnosis. Often, the diagnosis is delayed in
women because it is thought that the likelihood of a certain
disease process is rather low. Hopefully, this discussion
has helped to illustrate and highlight the structural heart
disease processes that are actually seen more frequently in
women.
REFERENCES
1. Dalen H, Thorstensen A, Vatten LJ, et al. Reference values and distribution of conventional echocardiographic
Doppler measures and longitudinal tissue Doppler velocities in a population free from cardiovascular disease. Circ
Cardiovasc Imaging. 2010;3;61422.
2. Lang RM, Bierig M, Devereux RB, et al. Recommendations
for Chamber Quantification: A Report from the American
Society of Echocardiographys Guidelines and Standards
Committee and the Chamber Quantification Writing
Group, Developed in Conjunction with the European
Association of Echocardiography, a Branch of the
European Society of Cardiology. J Am Soc Echocardiogr.
2005;18:144063.
1920
13. Shaw LJ, Bairey Merz CN, Pepine CJ, et al. WISE Investigators.
Insights from the NHLBI-Sponsored Womens Ischemia
Syndrome Evaluation (WISE) Study: Part I: gender
differences in traditional and novel risk factors, symptom
evaluation, and gender-optimized diagnostic strategies.
J Am Coll Cardiol. 2006;47(3 Suppl):S4S20.
14. Talbott E, Guzick D, Clerici A, et al. Coronary heart
disease risk factors in women with polycystic ovary
syndrome. Arterioscler Thromb Vasc Biol. 1995;15(7):
8216.
15. Citro R, Caso I, Provenza G, et al. Right ventricular
involvement and pulmonary hypertension in an elderly
woman with tako-tsubo cardiomyopathy. Chest. 2010;137
(4):9735.
16. Donohue D, Movahed MR. Clinical characteristics,
demographics and prognosis of transient left ventricular
apical ballooning syndrome. Heart Fail Rev. 2005;10(4):
3116.
17. Donohue D, Ahsan C, Sanaei-Ardekani M, et al. Early
diagnosis of stress-induced apical ballooning syndrome
based on classic echocardiographic findings and correlation
with cardiac catheterization, J Am Soc Echocardiogr. 2005;
18:1423.
18. Hurst RT, Prasad A, Askew JW 3rd, et al. Takotsubo
cardiomyopathy: a unique cardiomyopathy with variable
ventricular morphology. JACC Cardiovasc Imaging. 2010;
3(6):6419.
19. Haghi D, Athanasiadis A, Papavassiliu T, et al. Right
ventricular involvement in Takotsubo cardiomyopathy. Eur
Heart J. 2006;27(20):24339.
20. Verheugt CL, Uiterwaal CS, van der Velde ET, et al. Gender
and outcome in adult congenital heart disease. Circulation.
2008;118(1):2632.
1921
CHAPTER 79
Echocardiography in the Elderly
Gopal Ghimire, Navin C Nanda, Kunal Bhagatwala, Nidhi M Karia
Snapshot
Aorc Stenosis
Aorc Aneurysm
Aorc Dissecon
INTRODUCTION
Aging induces significant alteration in the structure and
function of the cardiovascular (CV) system. Although these
adaptive changes are physiological and asymptomatic,
they are at times difficult to distinguish from preclinical
disease. Furthermore, presence of coexisting CV diseases
can modulate these adaptive responses and complicate
the clinical picture. In this chapter, we aim to discuss the
CV peculiarities in elderly patients.
Coronary Stenosis
Prosthec Valves
1922
Fig. 79.2: Aortic valve sclerosis. Three-dimensional transesophageal echocardiography. Short axis cropping at the tip of the aortic
valve (AV) obtained from a parasternal long-axis data set shows
a large aortic orifice (arrowhead in the Movie clip 79.2) with no
evidence of stenosis. The AV leaflets are mildly thickened consistent with sclerosis. (LA: Left atrium; TV: Tricuspid valve). (Movie
clip 79.2).
Source: Reproduced with permission from Nanda NC, Hsiung MC,
Miller AP, Hage FG. Live/Real Time 3D Echocardiography. Oxford,
UK: Wiley-Blackwell; 2010.
1923
Fig. 79.3: Aortic valve sclerosis. Three-dimensional transesophageal echocardiographic reconstruction. Shows a thickened tricuspid aortic valve (AV) with no significant pressure gradient. (LA: Left
atrium; RA: Right atrium; RV: Right ventricle) (Movie clip 79.3).
Fig. 79.4: Aortic valve sclerosis. Three-dimensional transesophageal echocardiographic reconstruction demonstrates a mildly
thickened, obliquely oriented bicuspid aortic valve (AV) with raphe
and no significant gradient consistent with AV sclerosis. (LA: Left
atrium; RA: Right atrium; RV: Right ventricle). (Movie clip 79.4).
1924
AORTIC STENOSIS
Prevalence and Pathophysiology
AS in adults is caused by a calcific process involving a
normal trileaflet or congenital bicuspid aortic valve (BAV).
This calcific process can progress from the base of the
cusps to the leaflets, eventually causing a reduction in
leaflet motion and effective valve area without leading to
commissural fusion.29 The AV is more likely to be tricuspid
in patients in their eighth, ninth, and tenth decades of life,
and bicuspid in younger patients.3032 However, a study
revealed that three out of nine nonagenarians undergoing
surgical AV replacement had bicuspid valves.33 In the
Cardiovascular Health Study, AS was present in 2% of
the entire study cohort (adults 65 years), 2.6% of those
75 years or older, and 4% of those 85 years or older.
Changing demographic pattern in the western society and
changing epidemiology of rheumatic heart disease have
rendered calcific AS as one of the most prevalent valvular
diseases. AS currently is considered the most frequent
indication for valve replacement surgery, and the second
most common indication for cardiac surgery in older
adults, which is surpassed only by coronary artery bypass
grafting (CABG).34
Two-Dimensional Echocardiography
(Figs 79.5 to 79.7; Also see Fig. 78.11
in Chapter 78)
Echocardiography is an integral part of the evaluation of
AS in order to confirm the diagnosis, and assess its severity
and evaluate its upstream consequences on the left
ventricle (LV). Clinical signs and symptoms are of limited
use in distinguishing critical from noncritical AS due to
unsatisfactory sensitivity and specificity in the aged.35
Although cardiac catheterization is regarded as the gold
standard for evaluation of valvular dysfunction, it is invasive
and is associated with a higher risk of complications in the
elderly. In a single center study, 22 of 101 patients with
valvular AS who underwent retrograde catheterization
of the AV had focal diffusion-imaging abnormalities in
cranial MRI, a pattern consistent with acute cerebral
embolic events after the procedure; three of these patients
(3%) had clinically apparent neurological deficits.36 Thus,
cardiac catheterization in the elderly portends substantial
risk of clinically apparent cerebral embolism and frequent
silent ischemic brain lesions. Cardiac catheterization is
1925
Figs 79.5A to D: A 79-year-old man with exertional angina and syncope. Two-dimensional transthoracic echocardiography. On the
parasternal long-axis view (A) a calcified aortic valve with restricted opening is shown at the arrow. The maximal velocity across the
valve on continuous wave (CW) Doppler is 4.1 m/s (B) with a velocity time integral (VTI) of 84 cm, which correspond to a peak gradient
of 67 mm Hg and mean gradient of 35 mm Hg. The PW across the left ventricular outflow tract (LVOT) measured a velocity of 88.4 cm/s
across the LVOT (C); (D) The aortic valve area and the dimensionless index are calculated, both corresponding to severe aortic stenosis.
Notice that small errors in the measurement of the LVOT diameter will result in large errors in the calculated aortic valve area, while
the dimensionless index is unaffected by this measurement. Adapted from Adegunsoye A, et al. Echocardiography. 2011;28:11729.
1926
1927
Figs 79.7A to F: Mitral and aortic annular calcification in a 99-year-old patient. Two-dimensional transthoracic echocardiography.
(A and B) Parasternal long-axis view (A) and parasternal short-axis view (B) at the level of the aortic valve show heavy calcification
involving the aortic valve, and aortic and mitral annuli (1), mitral valve (2), and mitral subvalvular apparatus (3, 4); (C) Parasternal
short-axis view at the level of the mitral valve shows calcification involving the posterior mitral leaflet and mitral annulus (2); (D)
Apical four-chamber view showing calcification involving the ventricular septal muscle (arrow). Arrowhead points to a pacemaker in
the right atrium (RA); (E) Apical two-chamber view showing calcification involving the mitral valve and annulus (2). R represents a
reverberatory artifact deep in the left atrium from the calcified mitral valve. (F) Color Doppler-guided continuous wave Doppler interrogation of the aortic valve demonstrates peak and mean gradients of 30 and 18 mm Hg, respectively (arrow), consistent with mild
aortic stenosis. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RV: Right ventricle) (Movie clips 79.7A to E).
1928
The continuity equation provides an indirect rather than direct estimation of the AVA
True LVOT velocity can be difficult to determine from the area of increased flow acceleration
Accurately measuring LVOT diameter may be difficult (e.g. in patients with aortic or mitral annular calcification)
Doppler cursor may not be in the jet core even with color Doppler guidance, resulting in AS severity underestimation
Localized high gradients may be present in the region of the aortic valve; these do not reflect the true gradient across the AV,
resulting in overestimation of AS severity
AS severity cannot be correctly assessed in the presence of coexisting subaortic or supravalvular stenosis
Source: Adapted from Vengala et al.52
(AS: Aortic stenosis; AV: Aortic valve; AVA: Aortic valve area; LVOT: Left ventricular outflow tract).
1929
1930
Live/real time three-dimensional transthoracic echocardiography (3D TTE) with a full matrixarray transducer has
Figs 79.9A to C: Aortic valve stenosis. Live/real time threedimensional transthoracic echocardiography. (A to C) Careful
cropping of the parasternal long-axis data set at the flow-limiting
tips of the aortic valve (AV) leaflets demonstrated a bicuspid
morphology and severe stenosis. (LA: Left atrium; LV: Left
ventricle). (Movie clips 79.9A to C Parts 1 to 4).
Source: Reproduced with permission from Nanda NC, Hsiung MC,
Miller AP, Hage FG. Live/Real Time 3D Echocardiography. Oxford,
UK: Wiley-Blackwell; 2010.
1931
Fig. 79.11: Mild aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction showing a mildly calcified
tricuspid aortic valve (AV) with minimal stenosis. (LA: Left atrium;
RA: Right atrium; RV: Right ventricle) (Movie clip 79.11).
Fig. 79.12: Severe aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction. Demonstrates a heavily
calcified, vertically oriented bicuspid aortic valve (AV) with a very
small orifice indicative of severe stenosis. (LA: Left atrium; RA:
Right atrium; RV: Right ventricle) (Movie clip 79.12).
1932
Fig. 79.13: Severe aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction. Demonstrates a heavily
calcified, horizontally oriented bicuspid aortic valve (AV) with a very
small orifice denoting severe stenosis. (LA: Left atrium; RA: Right
atrium; RV: Right ventricle) (Movie clip 79.13).
Figs 79.14A and B: Severe aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction. (A) Shows a heavily
calcified tricuspid aortic valve (AV) with small a very small orifice consistent with severe stenosis; (B) In addition, a perforation
(arrowhead) is visualized in diastole in one of the cusps. (LA: Left atrium; RA: Right atrium; RV: Right ventricle) (Movie clip 79.14).
Fig. 79.15: Severe aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction. Demonstrates severe
tricuspid aortic valve (AV) stenosis with a very small orifice but
only mild calcification. (LA: Left atrium; RA: Right atrium; RV: Right
ventricle) (Movie clip 79.15).
1933
Table 79.2: Indications for Performing Three-Dimensional Echocardiography in Older Adults with Suspected Aortic Stenosis
Figs 79.16A and B: Severe aortic stenosis. Three-dimensional transesophageal echocardiographic reconstruction. (A) Demonstrates
a horizontally oriented severely stenotic bicuspid aortic valve (AV) with a valve area of 0.9 cm2; (B) Diastolic frame shows a perforation
(arrowhead) in the AV. (LA: Left atrium; RA: Right atrium; RV: Right ventricle; RVO: Right ventricular outflow tract) (Movie clip 79.16).
1934
1935
1936
Figs 79.18A and B: Aortic arch aneurysm with rupture. Live/real time three-dimensional transesophageal echocardiography. (A) The
top arrowhead points to the aneurysm in a 76-year-old male with a bioprosthetic aortic valve, which contains thrombus (T), and the bottom arrowhead denotes the site of rupture of this aneurysm into the mediastinum. Movie clips 79.18A Parts 1 and 2. In Movie clip 79.18A
Part 2, arrowheads in the right upper panel and in the left lower panel point to en face views of the aneurysm rupture site and mouth of
the aneurysm, respectively. Note the presence of spontaneous echo contrast suggestive of low blood flow state in the aneurysm and
aortic arch (ACH); (B) Ascending aortic aneurysm in a 67-year-old male with rupture into the mediastinum. Arrowhead points to a large
rupture visualized en face measuring 1.75 2.34 cm, area 3.01 cm2. It was not possible to visualize the rupture site en face by twodimensional echocardiography. Movie clip 79.18B.
Source: Reproduced with permission from Joshi D, Bicer E, Donmez C, et al. Incremental value of live/real time three-dimensional
transesophageal echocardiography over the two-dimensional technique in the assessment of aortic aneurysm and dissection. Echocardiography. 2012;29:62030.
The etiology of aneurysms can be degenerative, related to cystic medial degeneration (CMD), genetically triggered, atherosclerotic, inflammatory, traumatic, or mycotic. CMD is a common denominator in many genetically
triggered TAA including Marfan syndrome (MFS). In addition, normal aging is associated with some degree of CMD
and this process is accentuated by hypertension. These
changes cause progressive weakening of the aortic wall,
leading to dilation and aneurysm formation. The genetically triggered TAA can be syndromic with multisystem
manifestations [MFS, LoeysDietz syndrome (LDS), vascular EhlersDanlos syndrome (vEDS), Turner syndrome
(TS)] or nonsyndromic [familial TAA and dissection syndrome (FTAA/D), aortopathy associated with BAV] that
manifests with thoracic aortic disease alone.
The TAA in MFS involves the sinuses of Valsalva
with the ascending aorta above the sinotubular junction
usually being normal in dimension. TTE provides excellent
imaging of the aortic root and the sinuses of Valsalva and
is therefore an adequate imaging tool for evaluation and
surveillance of TAA size in MFS. In contrast, in LDS aortic
root, involvement is less common, with the descending and
abdominal aorta and aortic branch vessels more frequently involved. TTE alone may, therefore, not be sufficient
enough for adequate evaluation as well as surveillance
1937
Figs 79.19A to D
AORTIC DISSECTION
(FIGS 79.19 TO 79.22)
Although aortic dissection may occur in genetically
predisposed young adults such as those with MFS, LDS,
vEDS, FTAA/D, BAV, or TS, but it is mainly a disorder of the
1938
Figs 79.19A to G: Aortic dissection in an elderly patient. Twodimensional transthoracic and transesophageal echocardiography.
(A to G) Transthoracic parasternal long-axis (A) and apical fivechamber (B) views demonstrate irregularly moving linear echoes
(arrows) in the aorta (AO), resembling a wiggling worm typical of
aortic dissection. Thus, in some cases, a definitive diagnosis of
aortic dissection can be made by transthoracic echocardiography
precluding a transesophageal study, which can subsequently be
done in the operating room. This prevents undue delay in taking the
patient for surgery. In this patient, the dissection flap can be clearly
seen protruding into the left ventricular outflow tract in diastole and
this resulted in severe aortic regurgitation. Transesophageal long(C) and short-axis (D) views of the aortic root and ascending aorta
in another elderly patient show the dissection flap (arrow) extending into the right coronary artery (arrowhead). It is also seen in the
vicinity of the origin of left main coronary artery (LMCA) but does
not extend into it. Examination of the descending thoracic aorta in
short- (E and F) and long-axis (G) views show the perfusing lumen
(PL) surrounded by the nonperfusing lumen (NPL, arrow). Movie
clips 79.19E shows the dissection flap mimicking opening and closing motion of a persons mouth as if the patient was begging for
help, the so-called Help sign. (LA: Left atrium; LV: Left ventricle;
RV: Rright ventricle). (Movie clips 79.19A to E).
Figs 79.20C to H
1939
1940
I
elderly with ascending aortic dissection, most prevalent
between 50 and 60 years of age, and descending aortic
dissection peaking at 6070 years of age. Although the
classical presentation of the patient to the emergency
room is severe tearing chest pain radiating to the back
along with symptoms of autonomic activation, this
typical presentation may be modified by the location of
dissection and coexisting complications including acute
coronary syndrome (ACS). Aortic dissection and ACS can
present synchronously as the aortic dissection can cause
impairment to coronary flow due to a variety of reasons:
the dissection flap may mechanically obstruct the orifice of
the left or right coronary artery, the dissection process may
extend along the walls of a coronary artery significantly
narrowing the vessel lumen, subadventitial hematomas
(commonly present and alerts the surgeon to the presence
of dissection as soon as he opens the chest wall) may
compress the coronary arteries, localized pericardial
effusion either due to heart failure or partial rupture of the
dissected aorta into the pericardium may also compress
the coronaries, and finally, hypotension resulting from
dissection in an elderly patient with preexisting significant
CAD may precipitate severe myocardial ischemia and/or
myocardial infarction. It is important that the diagnosis
of acute aortic dissection be made emergently and if it
involves the ascending aorta and/or aortic arch (DeBakey
type I or type II dissection or Stanford type A dissection),
urgent surgical intervention is necessary. Any delay in
doing this substantially increases the mortality rate,
which in acute dissection is very high, up to 12% per
hour reported in the first several hours after dissection.118
This has led to the recommendation that even a coronary
arteriogram should not be performed in an elderly patient
1941
Figs 79.21A to C: Aortic dissection. Transesophageal threedimensional echocardiographic reconstruction. (A and B) The
descending thoracic aorta was examined using multiple cut sections
and various viewing angles. Both the true (TL) and the false (FL)
lumens are well visualized, and the dissection flap (F) presents as
a sheet-like structure along the aortic length. The communication
(arrows) between the true and false lumens is viewed en face using
a transverse section in C. (H: Mediastinal hematoma that resulted
from rupture of dissection).
Source: Reproduced with permission from Nanda NC, Khatri G,
et al. Three-dimensional echocardiographic assessment of aortic
dissection. Echocardiography. 1998;15(8):74554.
Figs 79.22A and B: Aortic dissection. Two-dimensional (A) and live/real time three-dimensional (3D) transesophageal; (B) echocardiography. Arrowhead points to a linear echo in ascending aorta (AO) consistent with dissection. However, instrument artifacts may present
as linear echoes in aortic lumen mimicking dissection. Therefore, in cases where a doubt exists as to the origin of the linear echo, it is
best to do a live/real time 3D study. The dissection flap will then appear as a sheet with finite width when viewed en face reflecting splitting of the aortic wall by the dissection process. Thus, aortic dissection can be confidently diagnosed or ruled out. (RPA: Right pulmonary
artery) (Movie clip 79.22).
1942
1943
ECHOCARDIOGRAPHY IN STROKE
PATIENTS: ASSESSMENT OF
CORONARY STENOSIS
(FIGS 79.23 TO 79.25; ALSO SEE
FIG. 78.13 IN THE CHAPTER 78)
Ischemic stroke constitutes 7080% of all strokes and
is caused by embolic or thrombotic occlusions of the
cerebral vessels and accounts for major morbidity and
mortality in the elderly.136 Embolic occlusions can be of
arterial or cardiac origin, with 1530% attributable to
cardioembolism (CE). Identification of the specific etiology
is crucial for risk stratification and in order to tailor the most
optimal preventive strategy. CE strokes portend a poor
prognosis with increased short- and long-term recurrence,
higher in-hospital mortality, and a higher index of fatal
recurrence versus other causes of stroke.136138 There is
no clear consensus on the indication and the optimal
echocardiographic approach in the cardiac evaluation
of ischemic stroke. The European Stroke Organization
guidelines recommend the use of echocardiography in
selected patients, while the American Stroke Association
guidelines do not make any clear recommendation on
its use..139,140 In real life practice, evaluation for cardiac
source for embolism is one of the most common requests
for the performance of a TEE (>25% of all studies at most
institutions).141 TEE identifies possible sources of CE
including the presence of a patent foramen ovale in >50%
of patients without clinically known heart disease, in
comparison, the diagnostic yield with TTE with agitated
saline-injection is only 25%, which drops to 10% without
saline-injection.142 However, the diagnostic superiority
of TEE does not necessarily translate into altered
therapeutic decisions. While the diagnosis of thrombus,
1944
Figs 79.23A to D: Ostial left main (LM) and mid-left anterior descending coronary artery (LAD) stenosis in a 72-year-old white male.
Two-dimensional transesophageal echocardiography. (A) Shows an area of flow disturbance in the ostium of the LM (1), preceded by
prominent flow convergence; (B) Color Doppler-guided continuous wave Doppler interrogation shows a very high diastolic velocity of
2.0 m/s (arrows) indicative of severe LM stenosis. Note also the high systolic flow velocity; (C) Demonstrates an area of aliased flow in
the region of mid-LAD (2) with a high diastolic flow velocity indicating significant stenosis; (D) Coronary angiogram showing 95% ostial
LM stenosis and 50% mid-LAD stenosis (arrows). (Ao: Aorta; Cx: Left circumflex coronary artery; LA: Left atrium; PA: Pulmonary artery)
(Movie clip 79.23).
Source: Reproduced with permission from Thakur A, Voros A, Nanda NC, et al. Transesophageal echocardiographic diagnosis of proximal coronary artery stenosis in patients with ischemic stroke. Echocardiography. 1999;16(2):15966.
Figs 79.24A to F
1945
1946
G
Figs 79.24A to G: Detection of left main (LM), mid-left anterior
descending coronary artery (LAD), and left internal carotid artery
stenosis and demonstration of atherosclerotic plaque in the left
subclavian artery in a 59-year-old black female. Two-dimensional
transesophageal echocardiography. (A) The black arrowheads
demonstrate prominent atherosclerotic plaques in the LM, producing 90% stenosis; (B) The arrow shows flow turbulence corresponding to the stenosis seen in A; (C) Color Doppler-guided continuous wave Doppler (arrow) demonstrates a very high diastolic
flow velocity of 3 m/s consistent with severe stenosis. The systolic
flow velocity is also high at 2 m/s; (D) The lower arrowheads point
to a prominent shadowing effect produced by the heavily calcified
plaque in the LM viewed in short axis (top arrowhead); (E) Color
Doppler-guided pulsed wave Doppler interrogation of the mid-LAD
(arrow) demonstrates a high peak diastolic flow velocity exceeding
1 m/s, indicating significant stenosis; (F) Withdrawal of the probe
into the upper esophagus and laryngopharynx demonstrates
marked narrowing of the proximal left internal carotid artery (LICA,
arrow), indicative of severe stenosis; (G) The arrow shows a large
soft plaque occupying 50% of the proximal left subclavian artery
(LSCA) viewed in an oblique axis. (Ao: Aorta; Cx: Left circumflex
coronary artery; LA: Left atrium; LCC: Left common carotid artery;
LEC: Left external carotid artery; LV: Left ventricle; PA: Pulmonary
artery; RVO: Right ventricular outflow tract).
Source: Reproduced with permission from Thakur A, Voros A,
Nanda NC, et al. Transesophageal echocardiographic diagnosis of proximal coronary artery stenosis in patients with ischemic
stroke. Echocardiography. 1999;16(2):15966.
1947
1948
PROSTHETIC VALVES
(FIGS 79.26 AND 79.27)
Prosthetic valves are common in geriatric population. A
suggested estimate of the prevalence of valve prostheses
ranged from 0.2 per 1,000 in those aged 44 and under
to 5.3 per 1,000 in those 75 years of age and older.165 3D
TTE has been shown to be superior to 2D TTE in the
evaluation of prosthetic valves, especially the mechanical
prostheses, since it allows for the visualization of both
leaflets simultaneously, which increases the confidence
in excluding significant abnormalities.166 Although the
current guidelines continue to be based on the unreliable
Doppler-derived pressure gradients for the assessment
of prosthetic valve dysfunction, 3D TTE is emerging as a
more robust tool.167,168
In conclusion, echo/Doppler techniques represent
the most useful and most cost-effective noninvasive
modalities in the assessment of CV disease entities in the
geriatric patient. In addition, these techniques are also
useful in monitoring various structural and physiological
changes that occur in the CV system with aging.
1949
1950
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1956
CHAPTER 80
How to do Echo for the
Electrophysiologist
Chittur A Sivaram
Snapshot
Left Atrium
Atrial Septum
Pulmonary Veins
INTRODUCTION
The sub-subspecialty of cardiac electrophysiology (EP) has
made tremendous strides and remarkable progress in the
last three decades. Several major therapeutic advances in
EP techniques have resulted in new treatment options,
capable of providing favorable clinical outcomes in patients
with recurrent arrhythmias. Such innovative therapies
in EP include ablation treatment of atrioventricular (AV)
nodal reentrant tachycardia, accessory pathways, and atrial
fibrillation (AF) as well as device therapy for primary and
secondary prevention in ventricular tachyarrhythmias.
Echocardiography is frequently performed in
rhythm disorders for the initial assessment as well as
during follow-up. The appropriate use of transthoracic
echocardiogram (TTE), transesophageal echocardiogram
(TEE), and intracardiac echocardiography (ICE) is
an important responsibility of cardiologists involved
in the ordering, performance, and interpretation of
echocardiographic modalities.1 The following description
will attempt at delineating steps for maximizing the yield
of echocardiography in patients with rhythm disorders
through a focused approach to echo imaging relevant to
the EP diagnosis under consideration. This chapter will
be predominantly centered on the use of TTE and TEE.
Devices
ECHOCARDIOGRAPHY IN SUPRA
VENTRICULAR TACHYCARDIA
Often the typical patient with supraventricular tachycardia
(SVT) has a structurally normal heart and consequently,
a completely normal echocardiogram. This is particularly
true in AV nodal re-entrant tachycardia (AVNRT).
However, several aspects of TTE have special relevance
to the preablation assessment in SVT. The preablation
TTE should carefully document chamber dimensions
including left atrial (LA) size and volume. It will be helpful
to the electrophysiologist to have the dimensions of
coronary sinus (CS) to facilitate catheters placement in
the CS during ablation. Dimension of CS may be obtained
from the apical four-chamber view of TTE with posterior
angulation of the transducer or with TEE in the lower
midesophageal location.
1958
Fig. 80.1:Parasternal long-axis view of transthoracic echocardiogram (TTE) showing dilated coronary sinus (CS) in the left
AV groove region (arrow). The descending thoracic aorta is seen
outside the pericardial layer while CS is intrapericardial. Dilated
CS can be the result of raised right heart pressures or persistent
superior vena cava (SVC) drainage into CS.
Fig. 80.2: Apical four-chamber view of transthoracic echocardiogram (TTE) with posterior angulation of transducer demonstrates
the dilated coronary sinus (CS).
1959
Fig. 80.3:Apical four-chamber view of transthoracic echocardiogram (TTE). Saline contrast injection from the left arm shows
opacification of coronary sinus (CS) and right heart chambers. The
appearance of contrast in CS prior to right heart chambers is diagnostic of persistent left superior vena cava (PLSVC) to CS.
Fig. 80.5:Parasternal long-axis view transthoracic echocardiogram (TTE), showing dilated right heart chambers in a patient with
Ebsteins anomaly. The anterior right heart was composed of right
atrium (RA) with its atrialized portion of right ventricle (RV).
Scanning Tips
1960
LEFT ATRIUM
In patients with arrhythmias, imaging of the left
atrium provides very valuable information to the
electrophysiologist, particularly if ablation therapies are
being considered. Dilatation of left atrium is frequently
seen in patients with atrial arrhythmias such as AF,
atrial flutter, and atrial tachycardia. Careful assessment
of LA size is critical. Traditionally, the anteroposterior
dimension of LA in systole measured using M-mode
echocardiography from the parasternal long-axis view of
TTE is the standard method for reporting LA size. However,
in many patients there is an obvious discordance between
LA size measured with M-mode and LA dimension in the
superior-inferior axis seen in the apical four-chamber
view of TTE. Recent studies have demonstrated a strong
correlation of both LA volume and LA volume index with
cardiovascular outcomes.3,4 Most of the commercially
available echocardiography machines have measurement
packages that permit calculation of LA volume using the
Simpsons biplane method during TTE. Care should be
taken to obtain stop frame images in systole that clearly
demonstrates the LA outlines in the apical four-chamber
and two-chamber views, as well as exclusion of LA
appendage and pulmonary veins (PVs) from the trace
contours.
In patients in atrial arrhythmias [AF, A flutter and
atrial tachycardias], a significant risk of thromboembolic
complications exists. Several findings on TEE correlated to
increased thromboembolic events have been described.
1961
Figs 80.9A and B: (A) Transesophageal echocardiogram (TEE) showing left atrial appendage (LAA; midesophageal probe position,
scan plane angle 54). Left upper pulmonary vein is seen adjacent to LAA, and it appears that a good demonstration of LAA has been
obtained; (B) With additional scan plane angle without any change in probe position, a large additional segment of LAA is now visualized. This underscores the importance of careful scanning of LAA with different scan plane angles to assess the complex shape of LAA.
Scanning Tips
1. Obtain M-mode measurements of LA size.
2. Obtain LA volumes from apical four- and twochamber views.
1962
Figs 80.10A to D:(A) Two-dimensional transesophageal echocardiogram. Arrowhead points to an echodense mass within the left atrial appendage (LAA) consistent with a thrombus; (B to D) Three-dimensional transthoracic echocardiogram. Within the LAA there are two echo densities noted. Sequential cropping shows both to be parts of pectinate muscles, which traverse
the LAA. The upper echo density (upper arrowhead) is larger because it represents a short-axis cut through two pectinate muscles virtually
in contact with each other. This most likely represents the thrombus seen on the transesophageal echocardiogram. The second
echo density (bottom arrowhead) is smaller because only one pectinate muscle is involved. (LUPV: Left upper pulmonary vein).
Source: Reproduced with permission from Karakus G, Kodali V, Inamdar V, Nanda NC, Suwanjutah T, Pothineni KR. Comparative
assessment of left atrial appendage by transesophageal and combined two and three-dimensional transthoracic echocardiography.
Echocardiography. 2008;25:91824.
ATRIAL SEPTUM
Abnormalities of atrial septum are occasionally seen in
patients undergoing ablation. Preprocedural assessment
1963
Figs 80.11A and B: (A) Transesophageal echocardiogram (TEE) showing a recanalized left atrial appendage (LAA) after surgical
exclusion; (B) Peak velocity by continuous wave (CW) Doppler was 3.4 m/s with a peak gradient of 46 mm Hg across the recanalized
LAA.
Procedures
requiring
trans-septal
puncture
(e.g. PV isolation for AF) often produce a left-to-right shunt
at the fossa ovalis region immediately postprocedure.
These shunts are invariably small and the defects close
spontaneously over time. ICE guidance is used in most
ablation laboratories for trans-septal puncture. If the
puncture enters the upper part of atrial septum, significant
hematoma might result due to entry into the Waterstons
groove (an extracardiac space between the two atria due to
in-folding of the atrial walls).
A frequent abnormality of the atrial septum in patients
undergoing AF ablation is lipomatous atrial septum.
Significant thickening of the atrial septum can occur due
to deposition of fat and this might be mistaken for an atrial
tumor. Typically lipomatous atrial septum spares the fossa
ovalis region. Lipomatous atrial septum is more often seen
in patients with obesity.
Scanning Tip
Immediately after AF ablation, there is often a left-toright shunt at the site of trans-septal puncture.
PULMONARY VEINS
The critical role played by firing from PV in the genesis of AF
has now conclusively been recognized. This mechanism is
the basis for ablative therapies in AF (PV isolation). Many
patients undergoing PV isolation for AF require additional
procedures including additional ablation for AF, A Flutter,
1964
Fig. 80.12: Transesophageal echocardiogram (TEE) from midesophageal level, employing 120 scan plane angle as well as torque
shows left upper and lower pulmonary veins (PVs).
Fig. 80.13: Transesophageal echocardiogram (TEE) from midesophageal level showing right upper, middle, and lower pulmonary
veins (PVs). Right upper PV is adjacent to superior vena cava.
1965
Figs 80.14A and B: (A) Stenosis of the right middle pulmonary vein (PV) after atrial tachycardia ablation. Significant aliasing of color
flow is seen at the ostial site of right middle PV; (B) Pulsed Doppler sampling from right middle PV shows marked increase in velocities
at the site of aliasing (peak diastolic velocity 1.3 m/s).
Figs 80.15A and B: (A) Transesophageal echocardiogram (TEE) showing focal aliasing of the left upper pulmonary vein (PV) ostium
after atrial fibrillation (AF) ablation; (B) Pulsed Doppler from the left upper PV shows a peak velocity of approximately 1.5 m/s in diastole
consistent with PV stenosis.
1966
ECHOCARDIOGRAPHY IN
VENTRICULAR TACHYCARDIA
Echo imaging with TTE provides highly meaningful
information in patients with ventricular tachyarrhythmias.
As such, imaging with specific diagnostic possibilities in
mind is called for in a patient with ventricular ectopy. Most
patients with ventricular arrhythmias have an anatomical
substrate (i.e. underlying structural heart disease).
Common substrates of ventricular arrhythmias
include:11
Coronary artery disease with scar from prior myoc
ardial infarction
Dilated cardiomyopathy
a. Noncompaction
Hypertrophic cardiomyopathy
Infiltrative diseases
a. Sarcoid heart disease
Arrhythmogenic RV dysplasia (ARVD)
Since ventricular tachycardia (VT) ablations require
placement of catheters in left ventricle (LV), the presence
of LV apical thrombi should be evaluated. Off-axis
scanning of the LV apex and the use of Definity contrast
are very helpful techniques for the demonstration of LV
apical thrombi. Some EP laboratories are also interested
in obtaining information about the degree of aortic arch
atherosclerotic burden since ablation of VT requires
manipulation of catheters across the arch of aorta.
The Table 80.1 lists the common conditions associated
with ventricular ectopy and the potential findings that
should be carefully looked at during echo imaging.
Table 80.1: Common Conditions Associated with Ventricular Arrhythmias and Associated Echocardiographic Findings
Disease
Characteristic Findings
Increased LV dimensions
Reduced fractional shortening
Reduced left ventricular ejection fraction
(LVEF; Simpsons biplane)
Localized scar (thinning, abnormal wall motion)
LV mural thrombi
Dilated cardiomyopathy
Increased LV dimension
Reduced fractional shortening
Reduced LVEF (Simpsons biplane)
Diffuse hypokinesis
LV mural thrombi
Noncompaction12
Increased LV trabeculations
Ratio of noncompacted to compacted segments > 2
Hypertrophic cardiomyopathy
Small LV cavity
Hyperdynamic LV function
Asymmetrical septal hypertrophy
Systolic anterior motion of mitral valve
Mitral regurgitation
Aortic valve midsystolic closure
LV outflow gradient (at rest and/or during provocation, e.g.
Valsalva maneuver)
Contd...
Increased right ventricular outflow tract (RVOT) dimensions
( 32 mm in the parasternal long-axis view, 36 mm in the basal
short-axis view)
Increased echogenicity of moderator band
Prominent branching pattern
Focal outpouching of RV free wall
1967
Contd...
Disease
Characteristic Findings
Arrhythmogenic RV dysplasia
13
ECHOCARDIOGRAPHY IN CARDIAC
IMPLANTABLE ELECTRONIC DEVICES
Cardiac implantable electronic devices (CIEDs) are indi
cated in a broad array of conditions including symptomatic
bradycardia (pacing), ventricular arrhy
thmias and
sudden cardiac arrest (defibrillators for primary and
secondary prevention), and severe heart failure [cardiac
resynchronization therapy (CRT)]. Echocardiography
provides significant information in patients undergoing
evaluation for CIEDs as well as when infection and
bacteremia complicate CIED therapy.
In the preprocedural selection of CIEDs, the task
for the echocardiographer is to demonstrate the major
features of the underlying structural heart disease and
to provide relevant quantitative measurements dictated
by the guidelines applicable in CIED therapy. Patients in
need of CIED therapy for primary or secondary prevention
of VT have underlying structural heart disease (coronary
artery disease, hypertrophic cardiomyopathy, or dilated
cardiomyopathy) and reduced ejection fraction (EF).
Thus, the preprocedural assessment should include
careful estimation of EF by Simpsons method. Selection of
patients for CRT is based on symptom level of heart failure
and QRS duration. The role of echocardiography in patient
selection for CRT is highly controversial and not supported
by strong evidence.
All patients with CIED infections require echo
cardiography to rule out infective endocarditis.15 This
is due to the fact that infection in CIED patients may be
restricted to the device pocket in which case the duration
of antibiotic therapy is shorter. Thus, TEE is required
REFERENCES
1. Douglas PS, Garcia MJ, Haines DE, et al. ACCF/ASE/
AHA/ASNC/HFSA/HRS/SCAI/SCCM/SCCT/SCMR 2011.
Appropriate Use Criteria for Echocardiography. J Am Coll
Cardiol. 2011;57(9):112666.
2. Corcoran SJ, Lawrence C, McGuire MA. The valve of
Vieussens: an important cause of difficulty in advancing
catheters into the cardiac veins. J Cardiovasc Electrophysiol.
1999;10(6):8048.
3. Abhayaratna WP, Seward JB, Appleton CP, et al. Left atrial
size: physiologic determinants and clinical applications.
J Am Coll Cardiol. 2006;47(12):235763.
4. Gabriel RS, Klein AL. Managing catheter ablation for
atrial fibrillation: the role of echocardiography. Europace.
2008;10 Suppl 3:iii813.
5. Karakus G, Kodali V, Inamdar V, et al. Comparative
assessment of left atrial appendage by transesophageal
and combined two- and three-dimensional transthoracic
echocardiography. Echocardiography. 2008;25(8):91824.
6. Kumar V, Nanda NC. Is it time to move on from twodimensional transesophageal to three-dimensional
transthoracic echocardiography for assessment of left atrial
appendage? Review of existing literature. Echocardiography.
2012;29(1):11216.
1968
12. Stanton C, Bruce C, Connolly H, et al. Isolated left
ventricular noncompaction syndrome. Am J Cardiol. 2009;
104(8):11358.
13. Marcus FI, McKenna WJ, Sherrill D, et al. Diagnosis
of arrhythmogenic right ventricular cardiomyopathy/
dysplasia: proposed modification of the task force criteria.
Circulation. 2010;121(13):153341.
14. Thakur RK, Klein GJ, Sivaram CA, et al. Anatomic substrate
for idiopathic left ventricular tachycardia. Circulation.
1996;93(3):497501.
15. Sohail MR, Uslan DZ, Khan AH, et al. Management and
outcome of permanent pacemaker and implantable
cardioverter-defibrillator infections. J Am Coll Cardiol.
2007;49(18):18519.
16. Le Dolley Y, Thuny F, Mancini J, et al. Diagnosis of cardiac
device-related infective endocarditis after device removal.
JACC Cardiovasc Imaging. 2010;3(7):67381.
CHAPTER 81
Echocardiography in
Life-Threatening Conditions
Rachel Harris, Elizabeth Ofili
Snapshot
Chest Trauma
Aorc Dissecon
INTRODUCTION
The uses of two-dimensional transthoracic and transesophageal echocardiography have proven vital in risk
stratification of patients who present with life-threatening
conditions.1 In evaluating patients with hemodynamic
instability, new or worsening heart murmur, or a recent
history of blunt or penetrating trauma, echocardiography
has become the mainstay in identifying those with
critical injuries in need of surgical or immediate invasive
intervention. Transthoracic echocardiography is both
sensitive and specific for identification of pericardial
effusion, pericardial injury, and peritoneal fluid in the
setting of anterior chest trauma.2 The advantages of
transthoracic echo are its bedside availability, noninvasive
characteristics, and ability to provide rapid information
regarding cardiac structure and function. Transesophageal
echo has greater sensitivity and specificity in evaluating
valvular and aortic pathology, prosthetic valves, interatrial
shunts, and cardiac sources of emboli but is more invasive
(Table 81.1).3
Debakey Classificaon
Air Embolism
Hypovolemia
CHEST TRAUMA
In 2010, among US persons aged 144, unintentional injury
was the number one cause of death.5 Cardiac injuries
are among the most lethal of thoracic trauma patients,
especially in penetrating injuries.6,7 In this same age
group, the majority of nonfatal hospital injury visits were
secondary to blunt force trauma.8 Although the majority of
these patients die in the field from their injuries, among
the survivors who present to the emergency department,
meticulous detail and a high index of suspicion must be
held for the hemodynamically stable patient who may
rapidly deteriorate post presentation.
1970
Table 81.1: Perioperative Indications for use of Transesophageal Echocardiogram in Trauma Patients
Category 1
Acute hemodynamic instability in which ventricular function and its determinants are uncertain and have
not responded to treatment
Unstable patient with unexplained hemodynamic disturbances, suspected valvular pathology, or thromboembolism
Immediate evaluation of patient with suspected thoracic aortic pathology: aneurysm, dissection, or disruption
Pericardial window procedures
Category 2
Category 3
Cardiac Tamponade
Echocardiography results in the diagnosis of cardiac
tamponade as well as assists in rapid management and
treatment, that is, echocardiographically guided pericardiocentesis with catheter drainage.10 Patients presenting
with hemodynamic instability post penetrating or blunt
force chest trauma should be emergently evaluated for
cardiac involvement, namely, myocardial contusion or
pericardial effusion.11,12
The clinical presentation of diaphoresis, dyspnea,
muffled heart sounds, and hypotension should warrant
further evaluation for pericardial effusion.11,13,14 Careful
evaluation using parasternal long- and short-axis,
apical four-chamber, and subcostal windows should be
undertaken as well as the use of M-mode.15 Evaluation of
diastolic right ventricular collapse can be demonstrated
as well as right atrial collapse/inversion. Diastolic right
ventricular collapse is more specific but less sensitive than
right atrial diastolic collapse.
While most cases of cardiac tamponade are immediate,
there are rare reported cases of delayed tamponade up to
70 days post injury (Figs 81.1 to 81.4).16
1971
Fig. 81.5: Tricuspid valve (TV) respiratory flow variation. (LV: Left
ventricle; RV: Right ventricle).
Cardiac Contusion
The definition of cardiac contusion is a histological
diagnosis. However, the clinical diagnosis relies on the
sum of echo findings, electrocardiogram (ECG), and
1972
Figs 81.6A and B: Parasternal short-axis view (apical level) in end diastole (A) and end-systole (B). Note the focal inferior wall akinesis
(arrows) and small pericardial effusion (PE) in a patient post trauma. (LV: Left ventricle) (Movie clip 81.6A and B).
1973
Vena contracta width > 0.7 cm with large central mitral regurgitation (MR) jet [area > 40% of
left atrium (LA) area] or with a wall-impinging jet of any size, swirling in LA
0.70
60
50
0.40
Source: Adapted from Bonow RO, Carabello BA, Chatterjee K, et al. ACC/AHA 2006 Practice Guidelines for the Management of
Patients With Valvular Heart Disease: Executive Summary. J Am Coll Cardiol. 2006;48(3):598675.25
Figs 81.7A to D: Short-axis at the level of the mitral valve showing a small ventricular septal defect (VSD) without (A) and with (B)
color (arrow). Three months later, the VSD was noted to be significantly larger, measuring 1.5 cm in diameter shown without (C) and
with (D) color (arrows). (LV: Left ventricle; RV: Right ventricle). Courtesy of Dr Robert Chisholm, St Michaels Hospital, Toronto, ON, with
permission.47
1974
AORTIC DISSECTION
TTE and TEE are both are indicated (Class I) in diagnostic
imaging of suspected aortic dissection.26 The type and
extent of the dissection should be determined.
DEBAKEY CLASSIFICATION
Fig. 81.10: Doppler evidence of severe mitral regurgitation (arrow;
Vmax, 4.67 m/s). (LA: Left atrium; LV: Left ventricle).
1975
Central jet, width > 65% left ventricular outflow tract (LVOT)
> 0.60
60
50
0.30
< 200
80
Source: Adapted from Bonow RO, Carabello BA, Chatterjee K, et al. ACC/AHA 2006 Practice Guidelines for the Management of
Patients With Valvular Heart Disease: Executive Summary. J Am Coll Cardiol. 2006;48(3):598675.25
Fig. 81.11: Aortic valve rupture. Long-axis view reveals flail right
coronary cusp (arrow) resulting in severe acute aortic regurgitation. (AO: Aorta; LA: Left atrium; LV: Left ventricle) (Movie clip
81.11).
1976
Left Parasternal
Ascending aorta
Apical
Ascending aorta
Subcostal
Ascending aorta
Right parasternal
Ascending aorta
Suprasternal Notch
Aortic arch
Paraspinal
Descending aorta
PULMONARY THROMBOEMBOLIC
DISEASE
Figs 81.15A and B: Descending aortic dissection with intimal flap (arrows). (FL: False lumen; TL: True lumen). Courtesy of Dr Anekwe
Onwuanyi, Morehouse School of Medicine, Atlanta, GA (Movie clip 81.15).
AIR EMBOLISM
Air embolism can result post multiple injuries including
blunt and penetrating chest trauma (Fig. 81.20). It may
result in air in the coronary arteries and Doppler findings
on echo consistent with air in cardiac chambers or major
arteries.39 When a patent foramen ovale is present, a very
small amount of air can result in paradoxical emboli.
Patients may present with hemodynamic instability,
respiratory distress, or postcardiac arrest. Physical exam
findings may be the appearance of cutaneous petechiae.
1977
HYPOVOLEMIA
Consideration of hypovolemia should be part of the
differential in patients with hypotension. The size of the left and
right ventricles are not reliable indicators of hypo-volemia;
1978
Figs 81.21A and B: Normal sized left ventricle (LV) with hyperdynamic state in hypotensive patient. End diastole (A) and end systole
(B). (Movie clip 81.21).
1979
Figs 81.23A and B: Apical views. Left ventricular (LV) apical mobile thrombus (arrow) in newly diagnosed congestive heart failure
(CHF) in a patient who suffered a stroke 2 weeks later on anticoagulation therapy. (MV: Mitral valve; RV: Right ventricle) (Movie
clips 81.23A and B)
SUMMARY
In life-threatening conditions, both two-dimensional
TTE and TEE are essential in rapid risk stratification, via
evaluation of cardiac structures and function, as well as
Fig. 81.24: Left atrial thrombus (LA: arrow) extending into left atrial remain a current noninvasive and more cost-effective
appendage (not shown) in a patient with severe mitral stenosis.
mainstay in diagnosis and treatment strategies.
(AV: Aortic valve) (Movie clip 81.24).
REFERENCES
valvular disease, intracardiac devices, postmyocardial
infarction, deep venous thrombosis, and malignancy more
commonly.
Transthoracic echo has a sensitivity of 9095% and a
specificity of 8590% for detection of LV thrombi where the
presence of thrombus was confirmed at surgery or autopsy
(Figs 81.23A and B).43,44 LV mural thrombi are more difficult
to diagnose when contrast agents are not used.45
Optimal views for imaging LV thrombi are apical
images that position the ventricular apex in the near field.
The mass should be visualized in at least two views to
1980
5.
6.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
21.
22. Wilson RF, Bassett JS. Penetrating wounds of the pericardium or its contents. JAMA. 1966;195(7):51318.
23. Moore EE. Traumatic ventricular septal defect. Surgery.
2007;142(5):7767.
24. Nguyen R, Ouedraogo A, Deneuville M. Gunshot wounds
to the chest with arterial bullet embolization. Ann Vasc
Surg. 2006;20(6):7803.
25. Bonow RO, Carabello BA, Chatterjee K, et al. ACC/AHA
2006 Practice Guidelines for the Management of Patients
With Valvular Heart Disease: Executive Summary. J Am
Coll Cardiol. 2006;48(3):598675.
26. Clouse WD, Hallett JW Jr, Schaff HV, et al. Acute aortic
dissection: population-based incidence compared with
degenerative aortic aneurysm rupture. Mayo Clin Proc.
2004;79(2):17680.
27. Mintz GS, Kotler MN, Segal BL, et al. Two dimensional
echocardiographic recognition of the descending thoracic
aorta. Am J Cardiol. 1979;44(2):2328.
28. Khandheria BK, Tajik AJ, Taylor CL, et al. Aortic dissection:
review of value and limitations of two-dimensional
echocardiography in a six-year experience. J Am Soc
Echocardiogr. 1989;2(1):1724.
29. Erbel R, Alfanso F, Boileau C, et al. Diagnosis and
management of aortic dissection. Recommendations of
the Task Force on Aortic Dissection, European Society of
Cardiology. Eur. Heart J. 2001;22:164281.
30. Erbel R, Oelert H, Meyer J, et al. Influence of medical
and surgical therapy on aortic dissection evaluated by
transesophageal echocardiography. Circulation. 1993;
87:160415.
31. Erbel R, Engberding R, Daniel W, et al. Echocardiography
in diagnosis of aortic dissection. Lancet. 1989;1(8636):
45761.
32. Kanojia A, Kasliwal RR. Recent advances in echocardiography of aortic disorders. Asian Cardiovasc Thorac
Ann. 1998;6:1537.
33. Lankeit M, Jimnez D, Kostrubiec M, et al. Predictive
value of the high-sensitivity troponin T assay and the
simplified Pulmonary Embolism Severity Index in
hemodynamically stable patients with acute pulmonary
embolism: a prospective validation study. Circulation.
2011;124(24):271624.
34. Torbicki A. Echocardiographic diagnosis of pulmonary
embolism: a rise and fall of McConnell sign? Eur J
Echocardiogr. 2005;6(1):23.
35. Goldhaber SZ, Visani L, De Rosa M. Acute pulmonary
embolism: clinical outcomes in the International
Cooperative Pulmonary Embolism Registry (ICOPER).
Lancet. 1999;353(9162):13869.
36. Nazeyrollas P, Metz D, Jolly D, et al. Use of transthoracic
Doppler echocardiography combined with clinical and
electrocardiographic data to predict acute pulmonary
embolism. Eur Heart J. 1996;17(5):77986.
37. Frmont B, Pacouret G, Jacobi D, et al. Prognostic value
of echocardiographic right/left ventricular end-diastolic
diameter ratio in patients with acute pulmonary embolism:
38.
39.
40.
41.
42.
43.
44.
45.
46.
47.
1981
CHAPTER 82
Lung Ultrasound in Cardiology
Luna Gargani, Eugenio Picano
Snapshot
Methodology
Pleural Effusion
Pulmonary Embolism
INTRODUCTION
Assessment of the lung has always been considered
off-limits for ultrasound, since it is standard textbook
knowledge that because ultrasound energy is rapidly
dissipated by air, ultrasound imaging is not useful for the
evaluation of the pulmonary parenchyma.1 However, in
recent years, lung ultrasound scan (LUS) has proved to be a
useful tool for evaluating many different acute and chronic
heart and lung disease conditions.2 It is especially valuable
because it is a very easy application of echography, far less
technically demanding than echocardiography,3 rapid to
perform and interpret, portable, repeatable, non-ionizing,
and independent of the cardiac acoustic window. Thus, it
is suitable for a quick but meaningful evaluation for both
in-patients and out-patients.
Pneumothorax
Limitations
1983
METHODOLOGY
1984
Treatment
The recognition, quantification, and monitoring of pulm
onary congestion is important for clinicians at all stages of
care of the HF patient. Accurate assessment of effectiveness
of medical treatment in reducing pulmonary congestion
is mandatory in these patients. Chest X-ray remains by
far the best and most frequently used screening test for
the detection and in-hospital follow-up of pulmonary
congestion, although showing the previously mentioned
limitations. Another way to monitor congestion is through
monitoring body weight. However, this has limited
reliability as a predictor of congestion status, since body
Prognosis
Persistent hemodynamic congestion that is not adequately
recognized and treated before discharge is associated with
adverse clinical outcome in HF patients; on the other
hand, postdischarge freedom from pulmonary congestion
is associated with a better prognosis.22 It has been shown
that in patients admitted to the hospital with dyspnea and/
or chest pain, the presence of B-lines identifies a subgroup
at a higher risk of experiencing eventsthe higher the
number of B-lines, the worse the outcome. The 16-month
event-free survival showed a significantly better outcome
for those patients without B-lines, whereas a worse outcome
was observed in patients with a severe grade of B-lines
(total number > 30). In regard to future HF hospitalization
alone, and not as part of the combined endpoint, the rate
of new hospitalization for progression of HF was higher in
patients with a severe B-line score and lower in patients
with no B-lines (log rank = 24.4, P < 0.0001).23 These data
have also been confirmed in a multicentric study on a
large number of patients on hemodialysis.24
1985
PLEURAL EFFUSION
Detection of pleural effusion (PE) is the more established
application of LUS.1 PE can be easily detected directly
through the intercostal spaces with either a cardiac or
convex probe. The effusion should first be sought in
dependent zones, that is, lateral and posterior chest. It
rules out other etiologies such as atelectasis, consolidation,
mass, or an elevated hemidiaphragm. It takes less time
than radiographic methods and can be repeated serially
at the bedside. Ultrasound images PE as an anechoic or
hypoechoic space between two pleural layers. The lung
behind a PE appears either aerated or consolidated in the
case of large PE. In critically ill patients, LUS is especially
valuable, showing better sensitivity and reliability than
bedside chest X-ray for the diagnosis of PE.25,26 Bedside
chest X-ray rarely detects small effusions and can also miss
effusions of up to 500 mL.27 For the detection of PE, with
computed tomography (CT) as a gold standard, sensitivity
and specificity of ultrasound are 93%.27 Minimal effusions
can be detected using ultrasound, provided the probe is
applied over an adequate area of the chest. Ultrasound
can detect the effusion, evaluate its volume, provide
information on its nature, and indicate the appropriate
area for an eventual thoracocentesis. Ultrasound is
acknowledged as the method of choice for detecting an
effusion in a supine patient.
PULMONARY EMBOLISM
The diagnosis of pulmonary embolism has always been a
considerable challenge and requires a high index of clinical
suspicion from the emergency physician. In addition,
diagnosing pulmonary embolism may require the use of
one or more direct and indirect diagnostic methods. Most
lesions related to pulmonary embolism are localized in
the lower lobes of the lung and are often associated with
an area of pleuritic chest pain. These processes essentially
involve the terminal air spaces and result in the evacuation
of air from the affected parenchymal area, creating an
ultrasonic window and allowing ultrasound waves to
travel further into the lung. The characteristic sonographic
findings in pulmonary embolism are multiple, hypoechoic,
pleural-based parenchymal lesions which adopt a wedge
shape.28 These lesions can be visualized at least in three
fourths of cases.29 The accuracy of LUS for the detection of
pulmonary embolism was found to be 84% at a prevalence
of 55% in a large multicenter study series comprising
352 patients with suspected pulmonary embolism.30
1986
ACUTE RESPIRATORY
DISTRESS SYNDROME
ARDS is a common syndrome of diffuse lung injury
and has a high mortality rate.32 In ARDS, LUS provides a
sensitivity of 98% and a specificity of 88% in diagnosing
the presence of the alveolar-interstitial syndrome as seen
at CT, performing better than either auscultation or chest
PNEUMOTHORAX
Pneumothorax (PTX) is a frequent, life-threatening
complication in patients who have been admitted to the
emergency department. Bedside chest X-ray may mis
diagnose up to 30% of cases.38 Radiographically occult
PTX may rapidly progress to tension PTX if its diagnosis
is missed or delayed, especially in patients receiving
mechanical ventilation.39 Being a nondependent
condition, PTX should be sought at first on the anterior
and lower areas. Absence of lung sliding is a basic and
initial step for the diagnosis, and a striking absence of
motion arising from the pleural line is observed instead
of the lung sliding.40,41 The presence of lung sliding allows
PTX to be confidently discounted because the negative
predictive value is 100%.40 The abolition of lung sliding
can be objectified in M-mode, which gives a characteristic
pattern, the stratosphere sign. However, absent lung sliding
does not necessarily mean PTX. Many other situations yield
abolished lung sliding, such as high-frequency ventilation,
massive atelectasis, pleural adherences, severe fibrosis,
and so on.5 The other conditions needed to diagnose PTX
by LUS are absence of B-linesthe slightest B-line allows
prompt ruling out of PTX.40 The pathognomonic LUS sign
of PTX is the lung point, which allows PTX to be confirmed,
with a specificity of 100% and sensitivity of about 65%, a
percentage that falls with major PTX with complete lung
retraction.42
1987
Table 82.1: The Three B-Line Scenarios: Heart Failure, ALI/ARDS, and Interstitial Lung Disease
Clinical Examples
Heart Failure
ALI/ARDS
Pulmonary Fibrosis
EF
Abnormal (decreased)
Normal
Normal
PASP
Abnormal (increased)
Normal
Normal/Increased
Effect of diuresis/dialysis
on B-lines
Reduction in minuteshours
No effect
No effect
Pleural effusion
Subpleural alterations
Pleural thickening
Theater
ER/Cardiology/Internal Medicine
Intensive Care
Pulmonology/Internal Medicine
(ALI: Acute lung injury; ARDS: Acute respiratory distress syndrome; EF: Ejection fraction; LUS: Lung ultrasound scan; PASP:
Pulmonary artery systolic pressure).
CARDIOPULMONARY ULTRASOUND:
AN INTEGRATED APPROACH
Echocardiography is an essential tool for the management
of patients with heart disease, providing an enormous
amount of information on both acute and chronic condi
tions. The addition of LUS to echocardiography provides
an additive insight into the presence of EVLW or any other
pulmonary involvement. Presence of multiple, diffuse,
bilateral B-lines associated with left ventricular systolic
and/or diastolic dysfunction or valvular heart disease is
highly suggestive of cardiogenic pulmonary congestion.
Moreover, for any given level of cardiac dysfunction,
the response of the pulmonary vascular bed may be
variable. LUS helps identify those patients who, although
asymptomatic, are going to decompensate and require
more aggressive treatment.43,44 Presence of multiple,
diffuse, bilateral B-lines, associated with a normal heart,
indicates a noncardiac cause of pulmonary edema such as
acute lung injury (ALI)/ARDS or interstitial pneumonia.
Alternatively, especially in a chronic setting, it should
prompt the suspicion of pulmonary fibrosis. It is important
to distinguish the multiple, diffuse, bilateral B-line pattern
from focal multiple B-lines, which can be present in
normal lungs or may be seen in the context of many
pathological conditions such as lobar pneumonia, pulmo
nary contusion, pulmonary infarction, pleural disease,
and neoplasia. This further underlines the importance of
integrating LUS findings with the patients history, clinical
presentation, and other instrumental data. An example
of the integration between clinical, echocardiography,
and lung ultrasound information for the differentiation of
the three main scenarios of B-lines in HF, ALI/ARDS, and
interstitial lung disease is reported in Table 82.1.
LIMITATIONS
LUS limitations are essentially patient-dependent. Obese
patients may be more difficult to examine due to the
thickness of their rib cage and soft tissues. The presence
of subcutaneous emphysema or large thoracic dressings
alters or precludes the propagation of the ultrasound
beams to the subpleural lung parenchyma. The main
limitation of B-line interpretation is the lack of specificity.
As previously mentioned, they are a sign of interstitial
syndrome; therefore, they are a very sensitive but not
specific sign of cardiogenic pulmonary edema. How to
distinguish the different etiologies of B-lines has been
discussed in this chapter. However, it must be emphasized
that LUS does not rule out pulmonary abnormalities
(especially consolidations) that do not reach the pleura.
In the next few years, LUS is likely to become an
extension of physical examination in many different
clinical settings, from the emergency department to the
intensive care unit, from cardiology to pulmonology, and
nephrology wards and dialysis units.
REFERENCES
1. Longo D, Fauci AS, Kasper DL, et al. Harrisons Principles
of Internal Medicine. 18th ed. New York: McGraw-Hill;
2011.
1988
1989
CHAPTER 83
The Future of
Echocardiography and Ultrasound
David Cosgrove
Snapshot
Trends in Scanners
Doppler
Microbubbles
Elastography
INTRODUCTION
Ultrasound has shown a remarkable ability to evolve
and even revolutionize, thanks to the efforts of research
laboratories and commercial companies around the
world. Unlike some industries, ideas developed in one
quarter find their way into clinical scanners surprisingly
rapidly, to the benefit of our patients. Here, some of the
more exciting and intriguing developments and prospects
are briefly covered, but it should be borne in mind that the
most important innovations may well be unpredictable:
expect the unexpected!
Fig. 83.1: Ultrafast plane wave imaging. The left image shows a
transverse section of a rat brain, measuring about 1 cm across,
imaged with the conventional pulse-echo technique. Spatial resolution is greatly increased by using plane wave imaging, shown
on the right, in which multiple interrogation at a different angle of
insonation (compounding) has been used.
Source: Redrawn with permission from Mace E, Montaldo G,
Cohen I, et al. Functional ultrasound imaging of the brain. Nat
Methods. 2011;8(8):6624.
1991
Fig. 83.3: Smartphone ultrasound system. This USB-based scanner from Mobisante uses a Smartphone to create ultrasound
images. The implications for usage and training are major. http://
www.mobisante.com/product-overview/
TRENDS IN SCANNERS
Miniaturization is well under way, with laptops and
even smart phones being used, if only for display5
(Fig. 83.3). These devices are likely to find their way into
every clinicians white coat pocket, supplementing or
even replacing their stethoscopes. This degree of miniatu
rization comes at a cost: their image quality cannot rival
that of high-end systems. This puts an extra burden on
1992
Fig. 83.4: Live/real time three- and four-dimensional transesophageal echocardiography. En face view of paravalvular mitral prosthetic regurgitation, oriented to be identical to the surgical view.
The paravalvular (P) defect is localized at 5 oclock position in
this patient with a metallic prosthesis. (AO: Aorta; LAA: Left atrial
appendage; MVR: Mitral valve replacement).
Source: Reproduced with permission from Singh P, Manda J, Hsiung MC, et al. Live/real time three-dimensional transesophageal
echocardiographic evaluation of mitral and aortic valve prosthetic
paravalvular regurgitation. Echocardiography 2009;8:9807.
1993
MICROBUBBLES
Fig. 83.6: Fusion imaging. In order to biopsy this liver lesion, a
previously acquired computed tomography (CT) scan has been
entered into the scanners memory and the two image sets aligned
using a position sensor attached to the ultrasound probe. The
lesion is highlighted in a green circle. This allows the complete
cross-sectional images of CT to be synchronized with the ultrasound images and the latter used for improved targeting of needles for biopsy and interstitial therapy.
DOPPLER
Doppler has lagged behind until recently. However, the
development of methods to mitigate its angle depen
dence should simplify its routine use. One method
uses compounding, so that each pixel or range gate is
1994
ELASTOGRAPHY
A special opportunity to examine the mechanical
properties of human tissue has been the development
1995
1996
THERAPEUTIC APPLICATIONS
OF ULTRASOUND
Diagnostic ultrasound is used at low powers, partly with
the intention of avoiding heating tissue, but if the power
is increased, heating occurs and this has been exploited in
physiotherapy. It is also effective for promoting healing of
bone fractures and skin ulcers.32
If much higher powers are used, tissue temperatures
can be raised to above 55C and the tissue coagulated.33,34
In this approach, a large transducer with tight focusing
concentrates the beam into a volume of about 2 10
mm; heating takes a few seconds and then the beam is
repositioned to the neighboring location and the steps
repeated across the desired volume that needs to be
ablated. The process is known as highly focused ultrasound
(HIFU) and is widely used, especially in China, to ablate
tumors, both benign and malignant as well as for pain
control in cancer of the pancreas.35 In many countries,
HIFU is officially recommended for prostate disease,
both benign hypertrophy and localized cancer. It has the
advantage of being noninvasive and very precise: the heat
coagulation completely spares tissue only a few cells away
from the coagulated region. Its disadvantage is that it is
slow, though ways to speed it up are being investigated.
Ultrasound is being developed as a means to allow
drugs to cross the skin barrier for transdermal drug
delivery.36 Low-frequency ultrasound transiently loosens
the squamous layer of the epidermis (probably by
cavitation) so that even large molecules such as insulin
CONCLUSION
The prospects for continued clinically significant develo
pments in ultrasonography are strong with many inno
vations in the offing. The area continues to surprise us with
innovations in diagnostics while therapeutic opportunities
are also on the horizon.
REFERENCES
1. Montaldo G, Tanter M, Bercoff J, et al. Coherent plane-wave
compounding for very high frame rate ultrasonography and
transient elastography. IEEE Trans Ultrason Ferroelectr
Freq Control. 2009;56(3):489506.
2. Shattuck DP, Weinshenker MD, Smith SW, et al. Explo
soscan: a parallel processing technique for high speed
ultrasound imaging with linear phased arrays. J Acoust
Soc Am. 1984;75(4):127382.
3. Tanter M, Bercoff J, Sandrin L, et al. Ultrafast compound
imaging for 2-D motion vector estimation: application to
transient elastography. IEEE Trans Ultrason Ferroelectr
Freq Control. 2002;49(10):136374.
1997
CHAPTER 84
A Primer on Cardiac MRI for the
Echocardiographer
Madhavi Kadiyala, Aasha S Gopal
Snapshot
Strain Assessment
Le Ventricular Structure
Cardiac Hypertrophy
Cardiomyopathies
INTRODUCTION
Significant progress has been made in the area of
cardiac imaging since the days of M-mode and B-mode
echocardiography. Advances in computed tomography (CT)
and magnetic resonance imaging (MRI) technology, along
with three-dimensional (3D) echocardiography enable the
modern cardiologist to make clinical diagnoses with high
accuracy and precision. Until recently, myocarditis was a
presumptive diagnosis in a patient presenting with elevated
cardiac troponins and normal coronary arteries. Today,
accurate diagnosis of myocarditis and the extent of myocardial
involvement can be made without invasive procedures using
cardiac MRI. This chapter discusses the practical applications
and limitations of cardiac MRI in various cardiac conditions.
A glossary of common cardiac MRI terms is included at the
end of this chapter.
While echocardiographic imaging is the first line of
diagnostic testing for most cardiac conditions, cardiac
MRI is valuable, when it is important to assess:
1. Tissue characterization: myocardial infarction, inflammation, and fibrosis
2. Accurate quantitation of cardiac chambers
Pericardial Disease
1999
Quantitative assessment of the right ventricle continues to be a challenge with echocardiographic methods.
Due to the complex morphology of the right ventricle,
no single view or imaging plane can provide adequate
information for accurate assessment of the right ventricle.
Although quantitative assessment by 3D echocardiography
is better that 2D-based methods,3 it is not widely available
and the technique needs further refinement before routine
use. Accurate right ventricular quantitation is particularly
important in arrhythmogenic right ventricular dysplasia,
atrial septal defects, anomalous pulmonary veins, etc.
It is standard practice to quantitatively determine RV
volumetrics by cardiac MRI. The inter- and intraobserver
variability by cardiac magnetic resonance (CMR) are very
low4 and allows for serial monitoring of chamber volumes.
Similar to the left ventricle, a short-axis stack of the right
ventricle is used for analysis (Fig. 84.1).
STRAIN ASSESSMENT
The complex myofiber arrangement in the heart allows it
to be a highly efficient pump, such that only 10% to 15%
single fiber shortening is required to generate an ejection
fraction of 65% to 70%. In the midwall, the myocardial
fibers generally have a circumferential layout. The myofibers become obliquely oriented in the endocardium and
epicardium, but in opposite directions, resulting in a perpendicular arrangement of the endocardial and epicardial
fibers. This arrangement leads to a finely orchestrated
deformation sequence in circumferential, longitudinal,
and radial directions. Circumferential shortening is primarily due to midwall fiber shortening. Longitudinal left
ventricular shortening is the result of contraction of the
Fig. 84.1: Epicardial and endocardial contours are semiautomatically traced on a stack of short-axis slices of the left ventricle in
end-diastole (shown) and end-systole. Endocardial area from each slice is measured and multiplied by the interslice distance. Thus,
accurate end-diastolic and end-systolic volumes are computed and ejection fraction is calculated (EDV ESV/EDV) and expressed as
a percentage. To obtain a time volume curve of the cardiac cycle, the endocardial contours need to be traced in all phases of cardiac
cycle.
2000
Fig. 84.2: Strain imaging by CMR. The left panel depicts regional myocardial deformation by harmonic phase analysis (HARP) of
tagged images. The green curve corresponds to the septum. The red curve corresponds to the lateral wall. The right panel depicts
regional myocardial deformation by feature tracking analysis of steady-state free precession (SSFP) cine images. Strain analysis of
seven segments in the four-chamber view is shown.
2001
Fig. 84.3: Left ventricular noncompaction (LVNC) in a young patient with recurrent episodes of syncope and history of atrial septal
defect repair as a child. 2D echocardiogram showed mildly reduced left ventricular function but missed the increased trabeculation, due
to suboptimal endocardial resolution. Contrast echocardiography was able to identify the abnormal myocardium, although in less detail
than CT or MRI. CT angiography demonstrated normal coronary arteries and increased trabeculations. Cardiac MRI was diagnostic
of LVNC. Pathological LVNC is often associated with congenital heart disease as in this patient; (CT: Computed tomography;
MRI: Magnetic resonance imaging).
2002
Figs 84.4A to C: LVNC: in addition to prominent trabeculations (A), deep recesses (B) are often seen in LVNC; (C) Physiological
hypertrabeculation: note the prominent apical trabeculation in C, which can be seen in healthy subjects. This does not indicate pathology.
Tissue Characterization
The ability to characterize tissues by using different
imaging sequences is a feature unique to MR imaging. The
sequences that are most commonly used are T1 weighted,
T2 weighted, fat suppression, and gadolinium-enhanced
sequences. Using various MRI sequences (Table 84.1), it
2003
Table 84.1: Signal Intensity (Brightness on the Images) of Various Tissues in the Common Tissue Characterization Sequences
Used in Cardiac MRI
SSFP
T1w
T2w
Gadolinium
Enhancement PSIR
Adipose
Suppressed
Fluid transudate
No
Fluid exudate
Int
Int
Int
No
Hemorrhagic
(acute) extra
cellular
methemoglobin
Hemorrhagic
chronic (deoxyhemoglobin,
intracellular
methemoglobin)
(low in
sub acute)
on early and
late imaging
Thrombus
(chronic)
Patchy fibrosis
Multiple patchy
foci in hypertrophic
segments (HCM > AS)
T1 mapping
techniques
Diffusely increased
SI irrelative to normal
myocardium
T1 mapping
techniques
Thrombus acute/
subacute
Infarction/
necrosis
None
No
Subendocardial/
Transmural Coronary
distribution
Collagen/amyloid
Calcification
TI scout
No
Iron
Vascularity
Other
Sequences
T2* sequence
(AS: Aortic stenosis HCM: Hypertrophic cardiomyopathy; PSIR: Phase sensitive inversion recovery; SSFP: Steady-state free precession).
Arrows indicate the signal intensity (SI) relative to myocardium. Details of the sequences are found in the text and the glossary.
2004
2005
Fig. 84.8: Myopericarditis: early and late gadolinium enhancement images both show multiple foci of hyperenhancement (green arrows)
in the myocardium, consistent with myocarditis. There is evidence of pericardial enhancement (blue arrows) indicating pericarditis. This
appears to be limited to the basal pericardium. The pericardium in the mid-sax images does not show enhancement. A moderate sized
pericardial effusion is present (star).
2006
Fig. 84.9: Cardiac sarcoidosis: intense hyperenhancement in the basal inferolateral wall on late gadolinium enhancement images in
this patient with history of pulmonary sarcoidosis and arrhythmias is suggestive of cardiac sarcoidosis.
CARDIAC HYPERTROPHY
Cardiac amyloidosis, hypertensive cardiomyopathy,
HCM, storage diseases such as Fabrys disease can all
cause cardiac hypertrophy and may be indistinguishable from one another by echocardiography. Tissue
characterization by MRI is useful in further evaluation
of these conditions.
Echocardiography is usually the reason to suspect
HCM with or without obstruction. Morphological features
of HCM, particularly the distribution of hypertrophy
(septal, midwall, or apical) are well characterized on
routine cine MRI images. Apical HCM may be missed
on echocardiography due to limited apical resolution.
Contrast-enhanced cardiac MRI images often, but not
always, show patchy hyperenhancement in areas of
2007
Fig. 84.10: Hypertrophic cardiomyopathy: severe hypertrophy of the septal myocardium with markedly abnormal LGE. Patchy areas
of enhancement with multiple foci in the thickened septum are indicative of extensive fibrosis. (LGE: Late gadolinium enhancement).
Fig. 84.11: Apical variant of hypertrophic cardiomyopathy: focal bright enhancement (blue arrows) in the apex on contrast-enhanced
image in a patient with apical HCM. Apical infarctions are also frequently observed due to coexisting microvascular disease. (HCM:
Hypertrophic cardiomyopathy).
2008
CARDIOMYOPATHIES
MRI offers additional information in the diagnosis of
cardiomyopathy beyond size and systolic function. The
role of MRI in dilated and other forms of cardiomyopathies
is discussed below.
DCM may occur in many conditions including viral
myocarditis, alcoholic cardiomyopathy, hemochromatosis,
thyroid disease, familial cardiomyopathy, etc. Most
commonly, DCM is idiopathic and is a diagnosis of
exclusion. It is believed that mechanisms specific to viral
2009
Figs 84.13A and B: Mid myocardial stripe pattern of enhancement on late gadolinium images is seen in this patient with dilated
cardiomyopathy.
2010
Fig. 84.15: Forward flow through the aorta (Qs) and pulmonary artery (Qp). In velocity- mapped images, pixels with velocity in either
direction are represented in grades of black or white (note the opposing colors in the ascending and descending aorta), whereas
stationary objects are represented in gray. The area under the curve is integrated to obtain the flow in either direction.
Aortic Stenosis
In general, direct planimetry of stenotic valve area is the
preferred method of assessing severity of aortic stenosis
by MRI. Valve area can be measured in either cine images
or in through plane velocity-mapped images and has been
shown to correlate well with Doppler echocardiography
and catheterization measures of severity.38,39 A crosssectional image through the leaflet tips in systole obtained
by carefully aligning two perpendicular left ventricular
outflow tract (LVOT) views is used to calculate the valve
area (Fig. 84.16). Peak and mean velocities can be obtained
by velocity mapping. Through planes that are perfectly
perpendicular to the stenotic jet at the tips of aortic leaflets
can be obtained, even in the setting of angulated aortic
roots.
It is also feasible to calculate aortic valve area by MRI
using the continuity method similar to echocardiography.
Through plane velocity maps of the LVOT and planimetered
area of LVOT are obtained by obtaining a perpendicular
plane through the LVOT and peak velocity obtained at the
leaflet tips is used to calculate the aortic valve area.
Aortic Regurgitation
Regurgitant jets are seen as flow void on steady-state free
precession (SSFP) images due to turbulence. There is a
modest correlation between the width of the jet and the
size of flow void with severity of regurgitation. However,
severity is often underestimated when using SSFP
sequences. It is often difficult to detect small amounts
of aortic regurgitation and may be missed by SSFP
imaging. Quantitative assessment by velocity mapping
is recommended for assessment of significant aortic
regurgitation.
Quantitative echocardiographic assessment of aortic
regurgitation (AR) can be challenging due to difficulty in
visualizing the vena contracta and proximal convergence
of AR jet. Assumptions involving mitral annular area
calculation limit the use of the continuity equation in
assessing AR. In contrast quantitative analysis by cardiac
MRI, velocity mapping is relatively straightforward.
Through plane flow is measured (Fig. 84.17) above the
2011
Fig. 84.16: Systolic frames of the aortic valve in long- and short-axis images are represented. The flow is in plane in long axis and
through plane in short axis. Aortic valve area can be traced by planimetry in either standard cine (SSFP) images or phase image
(as shown). In this example, it is easier to trace the contours on the phase image compared to the SSFP image. Respiratory artifacts
and dark signal from calcification render the SSFP image technically difficult to trace. (SSFP: Steady-state free precession).
aortic valve and below the level of the coronary artery ostia,
after carefully selecting the plane using two perpendicular
longitudinal views of the ascending aorta. Forward and
reverse flows are quantitated and regurgitant fraction is
calculated as follows.
Regurgitant fraction (%) = Aortic retrograde flow
(mL/beat) 100 Aortic forward flow (mL/beat).
The regurgitant volume may be underestimated by
velocity mapping due to the motion of the valve plane
during the cardiac cycle; however, interstudy reproducibility is high and the method is useful for long-term patient
follow-up.40 Quantitative assessment of left ventricular
volumes and function are equally important in long-term
follow-up. In the absence of other valvular regurgitation,
the difference between left and right ventricular stroke
volumes should equal the amount of aortic regurgitation
and this can be used to quickly corroborate the velocity
mapping analysis.
2012
Mitral Valve
When assessing mitral valve with cardiac MRI, standard
long-axis SSFP views are generally adequate for assessing
the motion and function of mitral valve. Systematic
evaluation of individual segments of mitral valve can
be done to identify prolapsed segments, when there is a
specific question of an anatomical abnormality (Fig. 84.18).
The resolution is, however, inferior to transesophageal
echocardiography, such that the anatomic detail is less
well appreciated. This is generally true with cardiac MRI
sequences, when assessing any thin structures that move
rapidly like valve leaflets.
Mitral Regurgitation
The major contribution of cardiac MRI in the setting of
mitral regurgitation is quantitative assessment of regur-
Fig. 84.18: Multiplane cine MRI images in a patient with posterior mitral valve prolapse and anterior regurgitant jet. By prescribing
multiple planes through a short-axis image of the mitral valve (last frame), it is possible to accurately localize the pathology.
(MRI: Magnetic resonance imaging).
Mitral Stenosis
In general, echocardiography is the preferred method
of assessing the anatomy and function of the mitral
valve in mitral stenosis. By prescribing through planes
perpendicular to the mitral leaflets it is possible to perform
direct planimetry of the mitral valve orifice. While velocity
2013
Tricuspid Regurgitation
Tricuspid valve anatomy can be assessed in the standard
long-axis views. Qualitative assessment of the tricuspid
regurgitant (TR) jet can be difficult because of less
prominent signal void in the setting of lower velocity
in the right ventricle (Figs 84.20A and B). Quantitative
analysis is useful and is performed using the same
principles as that of mitral regurgitation. The most
common method is
Tricuspid regurgitant volume = RV stroke volume
Pulmonary forward flow (mL/beat).
Quantitation of right ventricular volumes and function
is important in the evaluation of the severity of TR and for
long-term follow-up.
Prosthetic Valves
Despite the prevalent misconception that prosthetic valves
are a contraindication to cardiac MR imaging, almost
all prosthetic valves can be safely imaged at strengths of
1.5 tesla. It should be remembered that the mechanical
Fig. 84.19: Top panel demonstrates estimation of mitral inflow at the level of the mitral annulus. The area under the curve in diastole
yields the mitral inflow. Bottom panel depicts the flow pattern in the left pulmonary vein. The flow is systolic dominant.
2014
Figs 84.20A and B: Tricuspid regurgitation. The severity of valvular regurgitation is usually underestimated by SSFP sequences,
particularly in the case of right ventricle, where the regurgitant jets are of lower velocity. (SSFP: Steady-state free precession).
PERICARDIAL DISEASE
Echocardiography is the primary imaging modality to
evaluate pericardial disease; however, MR imaging offers
additional information that can help in clarification
of pathology and management. Cardiac MRI methods
allow for excellent tissue characterization, understanding
pericardial function, as well as presence of adhesions.
Normal pericardium is 1 to 2 mm thick (Figs 84.22A
and B) and is generally visualized as a layer of low signal
intensity on dark blood and SSFP images and is often
better visualized along the right ventricle, where there is
usually adipose between right ventricular free wall and the
pericardium. Tagged imaging can be helpful in assessing
slippage of the pericardium. In normal subjects, as
the myocardium contracts and relaxes, it slides past the
adjacent pericardium and slippage is clearly visualized
(Movie clip 84.3). In the setting of adherent pericardium,
the slippage sign is absent (Movie clip 84.4).
Differentiation of pericardial effusion from epicardial
fat can be difficult at times on echocardiography,
particularly if the adipose is extensive and has an atypical
2015
Figs 84.22A and B: Normal pericardium is identified by a layer of low signal between the layers of epicardial fat which has bright signal
on cine and LGE images. Pericardium along the right ventricle is better visualized.
Fig. 84.23: Pericardial cyst is noted adjacent to the left ventricle (). Fluid has bright signal on SSFP images and has a higher signal
on T2w (T2 > T1) images and short tau-inversion recovery (STIR) sequences. On phase sensitive inversion recovery LGE sequence,
fluid appears dark. In contrast, pericardial fat (blue arrows) has bright signal on SSFP, T1 weighted, and LGE images. Additionally,
signal from fat is suppressed on fat suppression and STIR sequences. (LGE: Late gadolinium enhancement; SSFP: Steady-state free
precession).
2016
Figs 84.24A and B: Pericardial thickening can be clearly seen on dark blood images along the left ventricle in this patient who had
features of constrictive pericarditis (Movie clip 84.6).
2017
Fig. 84.25: This patient was referred for cardiac MRI for evaluation of multiple right atrial masses on an echocardiogram. There is
extensive amount of epicardial fat that wraps around the right atrium along with lipomatous hypertrophy (blue arrows). Epicardial fat
in the tricuspid annulus often appears as right atrial mass. In addition, there is a prominent eustachian valve (orange arrows). The
eustachian valve is a normal atrial structure, but can be mistaken for a mass, when it is enlarged. A clue for identifying this correctly is
its location at the ostium of the inferior vena cava.
2018
Fig. 84.26: The mass noted in the right atrium on the echocardiographic images was demonstrated to be hypertrophic crista-terminalis
(blue arrows) and eustachian valve (orange arrows). Notice the thickened crista along the posterior wall of the right atrium that extends
from the inferior to the superior vena cava. This is well characterized on the serial cine planes through the atria.
Fig. 84.27: An unusually large thrombus seen in a young female patient with diabetes, peripheral vasculopathy, nephrotic syndrome
admitted with sepsis. Echocardiographic images in the upper panel demonstrate a large mass attached to the anterior wall, suspicious
for thrombus. There was severe biventricular dilation and systolic dysfunction. The absence of enhancement on perfusion and late gadolinium enhancement is consistent with thrombus. Additionally, there was diffuse hyperenhancement of the myocardium and abnormal
gadolinium kinetics consistent with cardiac amyloidosis.
2019
Fig. 84.28: Papillary fibroelastoma on the tricuspid valve. Note the low signal of the mass on the cine MRI (SSFP) images due to partial
volume effects. There is no evidence of fat suppression or perfusion. (MRI: Magnetic resonance imaging; SSFP: Steady-state free
precession).
CONCLUSION
Cardiac MRI has become a standard diagnostic modality
in todays cardiology practice, although availability of this
technique is still limited to a few major centers. It is the
gold standard in the determination of cardiac volumes
and evaluation of right ventricle, particularly when
2020
REFERENCES
1. Miller CA, Pearce K, Jordan P, et al. Comparison of
real-time three-dimensional echocardiography with
cardiovascular magnetic resonance for left ventricular
volumetric assessment in unselected patients. Eur Heart
J Cardiovasc Imaging. 2012;13(2):87195.
2. Mor-Avi V, Jenkins C, Khl HP, et al. Real-time
3-dimensional echocardiographic quantification of left
ventricular volumes: multicenter study for validation with
magnetic resonance imaging and investigation of sources
of error. JACC Cardiovasc Imaging. 2008;1(4):41323.
3. Gopal AS, Chukwu EO, Iwuchukwu CJ, et al. Normal
values of right ventricular size and function by real-time
3-dimensional echocardiography: comparison with cardiac
magnetic resonance imaging. J Am Soc Echocardiogr.
2007;20(5):44555.
4. Hudsmith LE, Petersen SE, Francis JM, et al. Normal human
left and right ventricular and left atrial dimensions using
steady state free precession magnetic resonance imaging.
J Cardiovasc Magn Reson. 2005;7(5):77582.
2021
2022
CHAPTER 85
Cardiac CT Imaging
Satinder P Singh, Sushilkumar K Sonavane
Snapshot
Radiaon Dose
Paent Selecon
Technique
Image Postprocessing
Image Analysis
Coronary Plaque
Tomography Angiogram
Cardiac Funcon
Myocardial Perfusion
Clinical Indicaons
Tomography Angiogram
INTRODUCTION
In the 1980s, the electron beam computed tomography
(EBCT) was introduced and was able to freeze cardiac
motion with a temporal resolution (TR) of 50 ms. EBCT
was used extensively for early coronary artery calcium
(CAC) evaluation. However, its role for CT angiography was
limited due to excessive noise and poor spatial resolution
(SR) from 3 mm to 4 mm thick images. Multidetector
computed tomography (MDCT), which was introduced
in the late 1990s, has rapidly evolved over the past few
years. A 64-MDCT is capable of providing TR of 165 ms
(85 ms with dual source scanner), SR of 0.4, slice thickness
of 0.6, and gantry rotation time of 330 ms or even less
(Table 85.1). MDCT can provide information about
coronary artery patency, coronary calcium, left ventricular
(LV) function, and to some extent even myocardial
perfusion. In addition, extracardiac sources of chest pain
2024
Table 85.1: Comparison of Spatial Resolution (SR) and Temporal Resolution (TR) for Different Modalities
Modality
> 0.6
50100
0.5
250400
64-MDCT
0.4
165250
Dual Source
0.4
83
Catheter Angio
0.2
520
0.7
20
Spatial Resolution
It refers to the ability to resolve as separate forms, small
objects that are very close together. Submillimeter SR with
isotropic imaging (i.e. equal resolution in all three planes)
is desirable to delineate small coronary artery branches.
The SR in CT depends on the size of the three-dimensional
pixels (voxel) in the image as seen on the monitor. The
smaller the size of voxel the less partial volume averaging
and better SR. The voxel size depends on the resolution of
the X-ray sensors and the focal spot size. It may also depend
Temporal Resolution
TR of a CT scanner is determined by the speed of rotation
of the gantry. Since images may be reconstructed from
a 180 rotation rather 360 rotation, the TR is equal to
half the gantry rotation speed. The current generation of
scanners has a very fast gantry rotation time of 0.270.30
ms. Excessive mechanical forces and G forces are the
limiting factors in further increasing the gantry rotation.
Two possible ways to improve TR are multisegment
reconstruction and use of dual source CT scanner.
Multisegment reconstruction selects small portions of
projection data from various heart cycles and combines
all projections to obtain sufficient data for image
reconstruction (TRmax = TR/2 M). However, variable
heart rhythm can cause image degradation due to
misregistration. Dual source CT design uses two X-ray
tubes and two detectors mounted on the gantry with a 90
angular offset and leads to high TR of 83 ms (one-fourth
rotation time; Table 85.1).
Cardiac Gating
Electrocardiography (ECG) gating of the tomographic
images is commonly performed in cardiac CT. Gating
not only minimizes cardiac motion artifacts and allows
2025
RADIATION DOSE
Figs 85.1A and B: (A) Nongated versus (B) Gated axial computed tomography (CT) image. Gated image showed minimal cardiac
motion related artifact, which helps in visualization of small size structures such as coronary arteries. (AO: Aorta; LAA: Left atrial
appendage; PA: Pulmonary artery).
2026
Modality
0.020.04
Mammogram
0.4
13
810
Abdomen CT routine
1012
Multiphase abdomen/pelvis CT
2234
Catheter Angio
37
SPECT
Coronary CTA
613 (retrospective)
15 (prospective), < 1 (High Pitch)
Thallium 201
2123
3.0
Average US background
3.64.5
Effective dose
E (mSv)
2027
PATIENT SELECTION
The revised 2010 appropriate use criteria for cardiac CT
were published in 2010.20 Use of noncontrast CCT for
calcium scoring is rated as appropriate in intermediate
and selected low-risk patients. CCTA is considered appropriate in: (a) Patients with suspected anomalous coronary
artery, symptomatic patients with low to intermediate
pretest probability for CAD who have normal ECG and
cardiac biomarkers, uninterpretable or nondiagnostic
ECG or equivocal biomarkers; (b) New onset heart failure
with reduced left ventricular ejection fraction (LVEF) in
low to intermediate probability for CAD and preoperative
coronary assessment prior to noncoronary cardiac surgery
and intermediate pretest probability; (c) Patients with
prior equivocal stress testing or when there is discordance
between the stress test and clinical suspicion for CAD;
(d) Evaluation for bypass patency after coronary artery
bypass graft (CABG) and prior left main (LM) coronary
stenting in asymptomatic patients; (e) Cardiac structure
and function category, with appropriate indications
including coronary anomalies, CHD, evaluation of right
ventricle (RV) function, evaluation of LV function when
imaging from other modalities is inadequate or evaluation
of prosthetic heart valves; (f ) Preablation pulmonary valve
(PV) mapping or prior to redo sternotomy in reoperative
cardiac surgery.
TECHNIQUE
Important steps to prepare patients for cardiac CT/
CTE are listed in Table 85.5. A radiologist or cardiologist
should monitor and supervise all CCTA studies for
optimization of protocol and best results. The type of
gating and technical parameters should be chosen based
on the clinical question asked, patient BMI, and heart rate
(Tables 85.6 to 85.11).
2028
For any C/I (renal failure, contrast allergy), note any pacemakers, details of bypass surgery
Oral -blockade
3 days preferred
IV access
Scanning Mode
Axial
kV
120
mAs
140240
Rotation time
0.5
Collimation
Scanning Mode
Helical
kV
80120
mAs
>600
Rotation time
0.4
64 .65
Collimation
64 .625
Pitch
0.2
Pitch
0.2
220
220
Filter
CB (standard cardiac)
Filter
Slice thickness
2.5 mm
Slice thickness
0.67 mm
Increment
2.5 mm
Increment
0.33 mm
Acquisition
Helical
Acquisition
Axial
IV contrast
Gating
Prospective
Saline chaser
40 cc at 45 cc/s
IV contrast
none
Gating
Retrospective
Effective Dose
13 mSv
Effective Dose
1015 mSv
Slice Thickness
1.4 mm
Increment
0.7 mm
Left atrium
Contrast
4080 cc at 4 cc/s*
Transaxial Images
IMAGE POSTPROCESSING
Complexity of coronary anatomy, cardiac motion, calciumrelated artifacts, and the subtle nature of coronary lesions
2029
Table 85.9: Transcatheter Aortic Valve Replacement (Retrospective Gated Chest and Nongated Abdominal/Pelvis Using Same
Contrast Bolus)
Contrast
60100 cc at 33.8 cc/s [depending on renal function and body mass index (BMI)]
Saline chaser
Bolus technique
Concentration
Phases
Bolus technique
Time bolus
Before
During
After
Multiplanar Reconstruction
A plane is defined inside the 3D volume, and only data
in this plane is displayed. It is performed by using either
straight or curved planes; thickness is set to zero as a
default to optimize image quality, but can be changed
(slab MPR). Its advantages include ease of use and speed,
provision of images containing all available information
(all Hounsfield unit values retained), usefulness in
delineating the morphology of the plaque, and its effect on
the lumen and adjacent vessel wall. Several disadvantages
include its operator dependence, prone to introduce false-
Maximum-Intensity Projection
This involves projection of highest-attenuation voxels
within volumetric data (all points below this value are
ignored). It uses thicker sections to include vessel lumen
and its wall (usually 45 mm for coronary), optimizes
visualization and tracking of contrasted structures and is
especially useful for blood vessel depiction (Fig. 85.5). The
advantages include: visualization of a longer segment of
a vessels course, reducing perceived image noise, good
differentiation between vessels and background, and
finally is useful when metal is present because of decreased
artifacts with this rendering. Loss of lesion information
within the slab volume (as MIP does not provide in-depth
information) and interference from overlapping structures
are its main limitations.
2030
Figs 85.2A and B: Double oblique method. A 54-year-old male with chest pain and abnormal stress MPT: Mixed plaque is seen in the
proximal left anterior descending (LAD) and more noncalcified plaque in mid-LAD after the origin of D1 (red arrow). Using the double
oblique method, the area of narrowing can be validated in three different planes.
Volume Rendering
This used to be a very tedious and time-consuming
process, but now with advanced fast computing powers
of modern processors this process is done with one or
few clicks. Due to its capacity to display multiple tissues
and their relationships to one another, VR is useful for
visualizing spatial relationships such as defining the
course of coronary anomalies, presence and course of
the bypass grafts as well as in the analysis of thoracic
CV structures and complex CHD (Figs 85.6A and B). VR
images are impressive and often used for teaching and
Virtual Endoscopy
In this technique, the dense contrast within the vessel is
made transparent while the wall of the vessel is opaque,
viewed from the point of view of an observer positioned
within the vessel. It creates dramatic images but its clinical
utility is limited at this time.
2031
Fig. 85.3: Curved multiplanar reformation (MPR). Centerline tracking allows visualization of the entire opacified vessel in one image.
Most current vendors provide semiautomatic centerline placement with one or two points of seeding.
Figs 85.4A and B: The centerline point shown in green is outside the vessel in image (A) causing an area of false stenosis on the curved
multiplanar reformation (MPR). After correctly placing the seed in the lumen of the coronary artery, the pseudostenosis disappears.
2032
Figs 85.6A and B: (A) Normal coronary artery. The frontal view demonstrates the most anterior chamber as the right ventricle and right
coronary artery coursing in the right artrioventricular (AV) groove (arrow). The broad pyramidal-shaped right atrial appendage (RAA) is
nicely seen. The aorta (AO) is to the right of the pulmonary artery (PA), which is more anterior and to the left of the aorta. (AO: Aorta;
PA: Pulmonary artery; RA: Right atrium; RV: Right ventricle); (B) The axial volume rendered image shows the anomalous origin of the
right coronary artery from the left cusp (red arrow) with interarterial course (yellow arrow). (L: Left cusp; LA: Left atrium; NC: Noncoronary;
PA: Pulmonary artery; R: Right cusp).
IMAGE ANALYSIS
Coronary artery evaluation should be done using the
standard AHA 17-segment evaluation. The coronary
arteries originate from superior portions of the sinuses
just below the sinotubular junction (Fig. 85.7). The normal
coronary artery origin is often situated at a right angle to
the aortic root wall, whereas anomalous coronary artery
origins are often at acute angles. The left coronary artery
originates from the left sinus of Valsalva and courses
between the right ventricular outflow tract (RVOT) and
the left atrial appendage (LAA). It typically divides into
2033
Extracardiac Findings
It is important for the imager to look at the entire FOV
for any given patient. In our experience we have found
unexpected lung nodules, lymph nodes, pulmonary
embolism, pneumonia, esophagitis, and aortic dissection
(Figs 85.15 to 85.18). In a study of 6,920 patients who
underwent diagnostic CCTA, the authors found presence
of extracardiac findings in almost one fourth of all patients.
2034
2035
Figs 85.11A and B: Coronary computed tomography angiogram (CCTA) 4 mm thick axial maximum-intensity projection (MIP) image
shows tight narrowing in the mid-left anterior descending (LAD; red circle), which was confirmed at catheter angiography. The outer wall
to outer wall size of the vessel at the site of noncalcified plaque is larger as compared to vessel proximal to stenosis suggesting positive
remodeling, which is one of the features of vulnerable plaque.
Figs 85.12A and B: In this young 47-year-old male with acute chest pain, the coronary computed tomography angiogram (CCTA)
demonstrates significant stenosis of the mid-left anterior descending (LAD) near the origin of the septal branch. The outer to outer size
of the vessel in this region is actually decreased in comparison to the proximal vessel suggesting the presence of negative remodeling.
Catheter angiogram confirmed the presence of fibrotic narrowing in the LAD.
2036
Figs 85.13A and B: Proper phase selection. In studies done using retrospective gating, the entire cardiac cycle images are available
from 0% to 90% RR intervals and can be reconstructed at 510% increment. Since cardiac motion is minimal in the mid to late diastole,
the left coronary circulation is best visualized during the diastolic phase as shown in these images. The right coronary artery is also seen
best in diastole except in a few instances where it is actually better seen at end-systole.
Figs 85.14A to C: Proper phase selection. RCA in 30%, 40%, and 80% phase. The last image also shows misregistration artifact
(arrow).
Blooming Artifacts
These occur with high attenuating structures such as
coronary stents or calcification due to partial volume
2037
Figs 85.15A and B: Extracardiac findings. In limited field of view (FOV) computed tomography angiogram (CTA), the right hilar and
subcarinal nodes (arrows) are barely visible at the edge, but become obvious with wide FOV image (B).
Figs 85.16A and B: The right lower lobe pulmonary embolisms (arrow) seen in wide field of view (FOV) image (B) can be missed
altogether if only limited FOV images (A) are reviewed.
Figs 85.17A and B: The nodule in the left upper lobe is difficult to appreciate in image (A) and can mimic an end on vessel. It is easy
to find on the wide field of view (FOV) image (B). The nodule in this patient was proven to be metastasis from renal cell carcinoma.
2038
Figs 85.18A and B: Extracardiac findings. A 45-year-old female presented with severe chest pain. A gated cardiac computed tomography (CT) was performed and showed normal coronary arteries but there was severe thickening of the entire thoracic esophagus
(arrows), which is better appreciated in wide field of view (FOV) image (B).
Figs 85.19A and B: Misregistration artifact due to variability in heart rate is better appreciated on coronal image (B, arrow) than the
axial image (A).
averaging and cause excessive blooming and overestimation of coronary luminal narrowing (Figs 85.21A
to C). A dual energy technique can provide images
without calcifications and may prove to be useful in better
assessment of coronary luminal narrowing by enhancing
vessel visualization. Similarly, monochromatic imaging at
different keV has been shown to decrease blooming from
calcium at 140 keV in comparison to lower keV.23 Coronary
stent-related artifacts can be improved by using a sharper
kernel for reconstruction as well as with thinner images,
although at the cost of slightly increased image noise
(Figs 85.22A and B).
Beam-Hardening Artifacts
An X-ray beam passing through a high-density structure
gets attenuated with most of its low energy photons
absorbed. In locations near such high attenuating
material, the X-ray beam maintains its low as well as high
energy photons, resulting in an area of low density in the
image. A common location for such an artifact is LV apex
and posterolateral wall of LV near the descending thoracic
aorta. It is important to recognize this artifact to avoid
misinterpretation (Figs 85.23A and B). Few vendors now
provide beam-hardening correction algorithms.
2039
Figs 85.20A and B: Respiration artifact. Breathing-related artifacts can degrade computed tomography angiogram (CTA) studies and
cannot be reversed with editing. These images are from the first patient scanned as gated computed tomography (CT) study on 40
detector scanner at our facility. The first image (A) showed a sudden cutoff of the mid-left anterior descending (LAD) which, of course,
was not seen at the subsequent cath. When you look at the lung window images at the same level, respiration motion-related artifact is
clearly visible as a ghost shadow in the pulmonary vasculature (arrow).
2040
Figs 85.22A and B: Stent evaluation. Sharper filter or kernel (B) often are very useful to look at the stent lumen and restenosis. The
images are very sharp and show the lumen, although at the cost of slightly increased noise in comparison to standard smooth filter
use (A).
Figs 85.23A and B: Beam-hardening artifact. The hypodensity noted near the LV apex (arrow) in this patient was not visualized on the
coronal images. This artifact can easily be misinterpreted for myocardial hypoperfusion.
DIAGNOSTIC ACCURACY OF
CORONARY COMPUTED
TOMOGRAPHY ANGIOGRAM
CCTA has become a robust and accurate clinical tool for
the noninvasive evaluation of the coronary arteries due
2041
angina, and found that in the ACS group the mean density
of the plaque was 25 15 H.U. whereas it was 71 16 H.U.
in patients with stable angina.42
In their study of plaque evaluation with CT attenuation
values, Sun et al. showed clear accurate characterization
of calcified plaques, but there was a significant overlap
between CT attenuation values of lipid rich and fibrous
plaques.43 The authors concluded that at this time, the
CT attenuation value is not able to accurately distinguish
between lipid-rich and fibrous plaques.
The relationship between coronary artery remodeling
and plaque composition has been studied and reported
by Varnava et al.44 These authors studied 88 male subjects
who died with CAD and examined 108 plaques postmortem; 59% had no or positive remodeling and 41% had
negative remodeling. They found that there was higher
lipid content and macrophage count in plaques with
positive remodeling (a marker of plaque vulnerability).
In another study, Reilly et al. looked at the effect of
statins on human coronary atherosclerotic plaque morphology.45 The authors retrospectively reviewed the arterial sections from native hearts of patients with end-stage
ischemic heart disease who received cardiac transplantation (Tx). Thirty-three of 44 study group patients received
pre-Tx statins, and 11 did not (which served as control).
Both groups were similar in total and low-density lipid
(LDL) cholesterol levels, and available number of arterial
sections per patient. The prevalence of low-grade fibrous
CORONARY PLAQUE
CCTA is excellent at detecting the presence of atherosclerotic plaques and shows good correlation with
intravascular ultrasound (IVUS).39 However, CTs tend
to underestimate the volume of a plaque. Leber et al.
compared plaque assessment by CTA to IVUS and found
that the vessel plaque volume is underestimated by
64-MDCT and its correlation with IVUS was only
moderate.40
Plaque Characterization
Figs 85.24A and B: Plaque characterization. Plaque characterization has been investigated and although lower computed tomography (CT) attenuation plaques are presumed to be lipid rich and those with CT attenuation between 40 and 120 are fibrous, there is
considerable overlap. At this time it is best to use terms calcified, noncalcified, or mixed plaque for CT descriptions. The CT images in
this Figure. belong to two different patients; (A) one with a positive remodeling has noncalcified plaque of CT values of 43, and (B) the
other has noncalcified plaque of CT value of 157 and associated negative remodeling.
2042
PROGNOSTIC INFORMATION
FROM CORONARY COMPUTED
TOMOGRAPHY ANGIOGRAM
The prognostic value of coronary calcification by CT has
been well described but the prognostic value of CCTA
is less well reported and remains a hot topic.46 Several
smaller studies have reported variable outcome from CTA
data.4756 In a recent meta-analysis of 18 studies evaluating
9,592 patients with a mean follow-up of 20 months, the
authors concluded that major adverse cardiovascular
events (MACE) among patients with normal CTA
are rare and there is incrementally increasing future
MACE with increasing CAD by CTA.46 In another study,
Russo et al. compared the prognostic value of CCTA with
that of coronary calcium scoring and clinical risk factors.57
The authors evaluated hard cardiac events in 441 patients
who underwent CCTA and calcium scoring for about
32 months. Patients with normal CCTA had a very low
hard annualized event rate of < 0.89%, in comparison
to 3.89% in patients with any CAD. Obstructive CAD
(P < 0.003) and presence of noncalcified or mixed plaque
(P < 0.0001) were independent predictors of hard events
on multivariate analysis.
In another study of 432 patients followed for 24 months,
van Werkhoven et al. looked at the incremental prognostic
value of CCTA compared with coronary calcium score
alone.58 In this study, multivariate analysis demonstrated
that the extent of disease and plaque characterization were
predictive of cardiac events, specifically plaque burden
and plaque composition provided incremental prognostic
value over clinical variables and coronary calcium scoring.
They also found that 20% of patients with a zero coronary
calcium score had noncalcified plaques and 4% patients
with no coronary calcium had significant CAD stenosis
(> 50%) by CTA, therefore suggesting that coronary
calcification alone may not be adequate to accurately
assess prognosis. James Min et al. looked at the prognostic
value of CCTA for prediction of all-cause mortality in
more than 1,127 patients older than 45 years.55 The patient
CARDIAC FUNCTION
LV function as reflected by the EF is one of the most
important prognostic parameters in patients with CAD. In
patients getting retrospective gating, LV assessment can be
easily performed with CT data available throughout the
cardiac cycle (Fig. 85.25).
In a study comparing cardiac function analysis from
MDCT with echo, single-photon emission computed
tomography (SPECT), and magnetic resonance imaging
(MRI), the authors found that there was agreement and
similar correlation between CT and MR for EF, enddiastolic volume (EDV), ESV, and LV mass parameters.59
The standard deviation of EF difference between CT and
MR was significantly less than that between echo and MR
or between SPECT and MR. The evaluation of RV function
is more challenging not only because of the shape of RV
but also often less than optimal contrast enhancement of
the RV when using CCTA protocol.
MYOCARDIAL PERFUSION
Although CT is currently not the method of choice to
evaluate myocardial perfusion, one can sometimes see
perfusion abnormalities in the myocardium in the resting
state (Figs 85.26A and B).
2043
Fig. 85.25: Cardiac functions. In retrospective gated CTA, cardiac function can be derived using CT threshold method and can be
displayed in bulls-eye or graphic representation.
2044
Figs 85.26A and B: (A) Resting myocardial perfusion abnormality. This 45-year-old-male came to the emergency department after
being involved in a motor vehicle collision. He had a routine chest/abdominal/pelvis computed tomography (CT). The representative
image of the chest CT shows a clear area of hypodensity in the mid to distal septum and apex with CT attenuation values of 33 in comparison to remote lateral wall LV myocardium with a CT value of 68. The wall thickness was maintained. These findings are suggestive
of acute myocardial ischemia/infarction and follow-up serial cardiac biomarkers and the electrocardiography (ECG) showed STEMI in
the left anterior descending (LAD) distribution; (B) In chronic myocardial infarction there is a subendocardial hypodense defect (arrows)
in a vascular territory associated with wall thinning and remodeling as shown in image (B).
CLINICAL INDICATIONS
Coronary Calcium Scoring
Quantification of CAC burden on CT is shown to be
an independent, noninvasive marker and predictor of
adverse cardiovascular (CV) events in asymptomatic
individuals.70 The risk assessment on CT CAC extends
beyond that provided by the Framingham risk score to a
population with a wide ethnic background.70 Thus, CAC
has the potential to restratify intermediate risk groups into
lower or higher groups.
Prospective ECG-gated cardiac images are acquired
with a slice thickness of 2.53 mm without intravenous
contrast. The FOV extends from just below the carina to
cardiac apex. Coronary calcium is defined as an area of at
least three adjacent pixels in the axial plane in the course
of a coronary artery, with an attenuation threshold value
of 130 H.U. or greater. Three in-axial-plane face-connected
pixels correspond to a minimum lesion area > 1 mm2,
which is used as a reference value in calcium scoring.71
There are currently two CT calcium scoring systems widely
used: the original Agatston method and the volume scoring
method developed by Callister et al.71,72 These images
are postprocessed on a dedicated 3D workstation with
appropriate software that automatically detects calcium
on all CT slices (Fig. 85.27).
2045
Despite recent advances in medical sciences, the evaluation of acute chest pain presenting in the emergency
department (ED) is difficult and remains a diagnostic
challenge. In a study of more than 10,000 chest pain
ED patients, 2.1% with acute MI and 2.3% with acute
unstable angina were inappropriately discharged.75 Due
to medicolegal issues, many of these patients with chest
pain get admitted unnecessarily for further tests and
investigations leading to billions of dollars of excessive
cost each year.76 In this scenario, a test with a very high
negative predictive value, such as CCT angiography, can
be very useful in effectively triaging this cohort of patients.
A major limitation, especially in acutely ill patients,
is the quality of the images especially if the patients have
tachycardia. Administration of -blockers, which is a
standard practice for CCT angiography, is time consuming,
is limited by contraindications, and may not be effective
in many acutely ill patients. Initial studies of dual source
CT have shown a very good diagnostic accuracy of CCTA,
even with a high heart rate.33,77
Improved assessment of coronary arteries with CT
and easy accessibility and close proximity of ED to such
scanners has increased the interest in MDCT evaluation
of chest pain. With 64-detector CT, sensitivity and
specificity rates of > 90% have been reported with few
nonevaluable segments than with earlier generation
MDCT scanners.28,30,78
A recent blinded prospective study of 103 patients
with chest pain who presented to the ED showed
absence of significant coronary stenosis and presence of
nonsignificant atherosclerotic plaque by MDCT, suggesting
absence of ACS, giving a negative predictive value of
100%.79 In another study, MDCT compared favorably
with radionuclide perfusion imaging for detecting ACS in
92 low-risk patients with chest pain in the ED.75
2046
Fig. 85.28: Coronary calcification. The cutoff coronary calcium is reported to be between 600 and 1,200. However, computed tomography angiogram (CTA) still could be useful if the calcification score for not performing CTA is limited to a few segments and it can
also show noncalcified lesions elsewhere as in these images. This patient had a calcium score of 1,060 causing mostly nonobstructive
disease, but there was additional more significant stenosis in the distal RCA due to mostly noncalcified plaque (red circle).
2047
2048
Fig. 85.31: RCA from left cusp. Sagittal oblique CT image shows
anomalous origin of the right coronary artery from the left cusp
and has interarterial course between aorta and pulmonary artery
(arrows). (L: Left cusp; NC: Noncoronary; PA: Pulmonary artery;
R: Right cusp).
Fig. 85.32: Volume rendering (VR) image clearly shows the empty
right cusp and anomalous origin of the right coronary artery from
left cusp. Due to the proximity of the anomalous vessel to commissure and proximal intramural course unroofing procedure can be
done in such cases with good results. (L: Left cusp; NC: Noncoronary; R: Right cusp).
Fig. 85.33: Anomalous origin of left coronary artery from pulmonary artery (ALCAPA). A 25-year-old female with increasing shortness of breath. Oblique volumerendered image demonstrates
the anomalous origin of the left coronary artery from the pulmonary artery (red arrow). The RCA (white arrow) is much larger.
2049
Preoperative Computed
Tomography Angiogram
When catheter angiography is considered dangerous,
difficult, or contraindicated, such as patients with aortic
vegetations, ascending aortic dissection and those with
massive aortic dilatation making it very challenging to
canulate coronary ostia (Figs 85.35A to C).
Role in Asymptomatic
Intermediate Risk Patient
Currently, CTA is not indicated for asymptomatic individuals and this is a topic of hot debate. One school of
thought leans toward getting CTA in patients with
significant risk factors including family history of premature atherosclerosis.
In a large multicenter registry study, Chinnaiyan
et al. presented their research data from the advanced CV
imaging consortium (ACIC) in Michigan looking at the
CCTA in asymptomatic individuals.88 A total of 21,573
patients without known CAD participating in ACIC at
43 institutions in Michigan were included and of these
11.8% were asymptomatic and 88.2% were symptomatic.89
The asymptomatic group patients were older, had more
males with higher Framingham risk scores, and higher
frequency of abnormal stress tests (38% vs 21%). CCTA
in these patients showed a lower frequency of normal
coronary arteries (38% vs 51.2%) and higher frequency
of both < 50% and > 50% luminal stenosis. The authors
concluded that the asymptomatic patients had worse risk
profiles and a higher CAD burden on CCTA. These findings
have broad implication on identification of candidates for
aggressive secondary prevention.
The main hurdle in our view has been the issue of
radiation exposure, which is understandable. But with
the recent advances and availability of very low dose CTA
protocols it seems logical and appropriate to use CTA
selectively in such high-risk populations with radiation
exposure close to or < 1 mSv. In fact, with such low-dose
scanning capability, it is possible that in the near future
2050
2051
Figs 85.36A and B: Redo sternotomy. Performing computed tomography (CT) is an appropriate indication for a patient being evaluated
for redo CABG or noncoronary cardiac surgery. CT can accurately detect the distance of vital structures near the sternum as well as the
presence of any adhesions. In the images here, this patient had developed a pseudoaneurysm (PSA) at the site of aortic cannulation
(arrows) done for cardiopulmonary bypass. Computed tomography angiogram (CTA) clearly showed the close proximity of the PSA to
the sternum, which is useful information for the surgeons in planning a safe surgical approach.
Cardiac Masses
Primary cardiac tumors are rare but metastases to the
heart from other locations are much more common.
Primary benign tumors are much more common than
primary malignant neoplasms. Myxoma is the most
common primary benign cardiac neoplasm in adults.
Most myxomas arise in the LA near the fossa ovalis and
less commonly from the atrial free wall or atrioventricular
valve. Less commonly they can arise in the RA or RV. On
CT, myxomas are round, smooth, and lobulated masses
often with a pedicle and demonstrate patchy postcontrast
enhancement. Often these masses have areas of calcifications as well as low attenuation (Fig. 85.40). On a
retrospective gated CT, the pedunculated myxoma can be
seen to prolapse through the mitral valve. Symptoms from
myxoma are usually due to hemodynamic obstruction,
embolism, or rarely constitutional symptoms. Multiple
cardiac myxomas can be associated with pigmented skin
lesions, extracardiac tumors (schwannoma, pituitary adenomas), and endocrine abnormalities (Carneys complex).
Lipoma is the second most common primary benign
cardiac tumor. It can either arise from the subendocardial
layer or subepicardial/myocardial layer. The endocardial
2052
Figs 85.37A to C: Coronary artery bypass graft (CABG) volumerendering (VR) image. (A and B) Proximal and distal anastomosis
(circle) with good distal flow from LIMA to posterior descending
artery (PDA) graft; (C) Multiple grafts are seen including left
internal mammary artery (LIMA) and two venous grafts. Metal clips
are actually not causing much metal streak artifact. (LSC: Left
subclavian artery).
Figs 85.38A and B: Coronal oblique computed tomography angiogram (CTA) image (A) shows the opacified LIMA with patent distal
anastomosis with diagonal branch (circle) and distal flow. The corresponding cath angio view (B) confirms these findings.
2053
2054
Figs 85.41A and B: Lipoma. A large fat-computed tomography (CT) density mass (arrow) is seen in the right atrium extending up to
the superior vena cava (SVC) to right atrium (RA) junction superiorly as seen in image (B). (AO: Aorta; LV: Left ventricle; PA: Pulmonary
artery).
2055
Figs 85.43A and B: Metastasis. Metastasis from melanoma involving the pericardium (star) aortic root and interventricular septum
(arrow).
Figs 85.44A and B: Angiosarcoma. Angiosarcoma of the heart (star) involving right ventricle (RV) and extending across the right
atrioventricular (AV) to involve the right atrium (RA).
Figs 85.45A and B: Pericardial cyst. A well-circumscribed water density nonenhancing mass is seen along the right atrium (RA).
2056
Figs 85.46A and B: Lipomatous hypertrophy of the interatrial septum. Lipomatous hypertrophy of the interatrial septum often spares the
fossa ovalis and produces a dumbbell-shaped appearance as shown in image (red circle). The fatty component can extend into the right
atrial wall to a variable degree and can sometimes encroach on the cavoatrial junction (arrow), although usually without any hemodynamic
consequences. (AO: Aorta; LA: Left atrium; LV: Left ventricle; RA: Right atrium: RV: Right ventricle; RVOT: Right ventricle outflow tract).
2057
Transcatheter Aortic
Valve Replacement
Transcatheter aortic valve replacement (TAVR) is rapidly
becoming a popular alternative procedure for older
patients with severe symptomatic aortic stenosis, who are
at high risk for open surgical repair. Several prosthetic
valves are available including the balloon expandable
Edwards Sapien Valve (Edwards Lifesciences Inc, Irvine,
CA, USA) in 23, 26, and 28 mm sizes and self-expanding
CoreValve (Medtronics Inc, Minneapolis, MN, USA)
available in 23, 26, 29, and 31 mm sizes. In the United
States, only the 23- and 26-mm Edwards valves are
currently approved. The most common implantation route
is transfemoral. If this is not feasible, the Edwards Sapien
valve can be implanted via direct transapical approach or
rarely via a transaortic approach. Currently, this system is
not approved for subclavian artery approach.
Imaging is necessary before the procedure to determine annulus size, annulus area, degree of aortic calcification, access route, and suitable fluoroscopic projection
angles that permit exact orthogonal views onto the valve.
Preprocedural assessment of the aortic root for TAVR
requires knowledge of the orientation of the aortic root
relative to the body axis. For correct deployment of a
percutanous valve prosthesis, the root orientation is
usually assessed by repeated X-ray aortograms in one
or two orthogonal planes. To simplify the procedure, 3D
data sets obtained by MDCT may allow the prediction of
angiographic projections orthogonal to the valve plane
(Figs 85.50A to C).
Figs 85.48A and B: PV ablation. Computed tomography angiogram (CTA) images are loaded into PV ablation software in the cath lab
and are fused with cath images to show the catheter location in relation to the left atrium (LA) and its veins. The electrical map is then
generated and shows as red dots (B), which need to be ablated. (LIPV: Left inferior pulmonary vein; LSPV: Left superior pulmonary vein;
RIPV: Right inferior pulmonary vein; RSPV: Right superior pulmonary vein).
2058
2059
2060
Figs 85.51A to D: Atrial septal defect (ASD) and Eisenmenger syndrome. Chest radiograph (A) shows an enlarged heart, dilated pulmonary arteries, and shunt vascularity. Axial computed tomography (CT) image (B) shows a large septum secundum defect (circle) along
with a dilated right atrium. The main pulmonary artery is dilated (C) and there is mosaic perfusion in both lungs (D). Findings are suggestive of secondary pulmonary HTN. (LA: Left atrium; LV: Left ventricle; RA: Left atrium; RV: Right ventricle; MPA: Main pulmonary artery).
Figs 85.52A and B: Sinus venosus atrial septal defect (ASD). A connection is seen between distal superior vena cava (SVC) and roof
of the left atrium (A, red arrow) along with anomalous right upper lobe (RUL) pulmonary vein drainage into the SVC (B, yellow arrow).
(AO: Aorta; LA: Left atrium; RA: Right atrium; RV: Right ventricle; RAA: Right atrial appendage; PA: Pulmonary artery; PV: Pumonary
valve).
2061
Figs 85.54A to D: Transposition of great arteries (TGA) status post (s/p) Senning operation. The pulmonary artery (PA) is to the left and
slightly posterior to the aorta (AO). It is connected to the morphological left ventricle (LV). An intra-atrial baffle (B) is connected to the
LV. The aorta is connected to the morphological right ventricle (RV), which is hypertrophied and dilated. The morphological left atrium
is surgically connected to the RV, thereby correcting the blood flow: systemic venous blood flows via the baffle (star) into the LV then
into PA and lungs. The oxygenated blood returns via the pulmonary veins into the LA and is directed into the RV and then into the aorta.
2062
Tetralogy of Fallot
Because the surgical repair is usually performed during
early childhood, the role of CT in diagnosing TOF is
minimal. In postsurgical evaluation, the main purpose
of CT is to visualize extracardiac complications, to depict
the morphological characteristics of the main PA and its
branches (to identify any obstruction, distortion after
previous palliative shunt creation, or aneurysm), and to
detect any right ventricular enlargement due to chronic
volume overload in the presence of severe pulmonary
regurgitation (Figs 85.56A and B).
Figs 85.55A and B: Corrected transposition. In congenitally corrected transposition, there is atrioventricular and ventriculoarterial discordance such that the left atrium (LA) is connected to right ventricle (RV), which is connected to the aorta. The right atrium (RA) is connected
to left ventricle (LV) and is then connected to the pulmonary artery (PA). The circulation circuit in these patients is: systemic blood return
morphological RA
morphological LV
pulmonary arteries. Oxygenated blood from lungs return via pulmonary veins
morphological LA morphological R V aorta.
2063
Figs 85.56A and B: Tetralogy of Fallot (TOF) after palliation treatment. A 64-year-old female with prior BlalockTaussig palliation for
tetralogy of Fallot. Oblique coronal three-dimensional (3D) volume-rendered reconstruction demonstrating right ventricular (RV) hypertrophy, overriding aorta (AO): and subaortic ventricular septal defect (VSD; asterisk). 3D volume-rendered slab reconstruction illustrating
the right ventricular outflow tract obstruction (arrow). (PA: Pulmonary artery).
Fig. 85.58: Left ventricular assist device (LVAD). Typical location of inflow cannula (A) in left ventricle (LV) midcavity, pointed
forward the mitral valve. The outflow cannula (B) is connected to
the ascending aorta.
There has been a steady increase in the number of continuous flow left ventricular assist devices (LVAD) implanted
annually, since the Heartmate II LVAD was approved for
bridge to transplant in 2008 and destination therapy in
2010. Given the persistent donor shortage and improvements in LVAD technology, patients are increasingly likely
to undergo LVAD placement and remain on LVAD therapy
for prolonged periods of time. Persistent left heart failure,
2064
Figs 85.59A and B: Outflow cannula thrombosis (B) in this patient with chronic heart failure and post-left ventricular assist device
(LVAD) complication. This was also seen in the aortic root injection angiogram done in an attempt to remove the thrombus.
contains 1535 mL of serous fluid.105 The normal pericardial thickness on CT/MR is < 2 mm.106,107 On CT, it is a
linear isodense structure between the epicardial and
pericardial fat.
Pericardial Diseases
Pericardium is a dual layer fibrous sac surrounding the
heart with inner visceral layer adherent to the epicardium
and the outer parietal layer. The potential space between
these two layers is called the pericardial cavity that
Pericardial Defect
It is a rare anomaly with the spectrum ranging from a
small defect to complete absence of the pericardium.110
It is commonly seen along the left side of the heart and
may have associated congenital abnormalities such as
ASD, PDA, or a bicuspid AV.111
The lack of visualization of normal pericardium is a
direct sign. However, it is difficult to visualize the normal
pericardium along the left side of the heart. The indirect
signs include levorotation of the heart, and interposition
of lung tissue between the aorta and main PA or between
inferior border of heart and diaphragm. Excessive motion
of the cardiac apex is seen on cine MR images.112 Complete
2065
Constrictive Pericarditis
Pericardial Effusion
Pericardial effusion may be transudate, exudates, or
hemorrhage. The role of imaging is to determine its
presence, pericardial thickening, and mediastinal or
thoracic abnormalities to explain the cause. There are no
specific criteria for distinguishing physiological versus
pathological pericardial fluid. Visual evaluation and
categorization as mild, moderate, or severe is routinely
performed (Fig. 85.61). Pericardial thickness of > 4 mm is
considered abnormal.113 Mediastinal lymphadenopathy or
presence of thoracic malignancy may raise the suspicion
for malignant effusion. Hemorrhagic effusions are seen
in the setting of trauma, dissection, postsurgery, and
will show CT attenuation value between 40 and 80 H.U.
(Fig. 85.62).
Pericardial Calcification
Calcification of pericardium is secondary to a prior insult.
Postcardiac surgery, hemopericardium, exudative effusions
such as tuberculous, postradiation are some of the common
causes of pericardial calcifications (Fig. 85.63). Pericardial
calcification may result in constrictive physiology and
Pericardial Tumors
Primary pericardial tumors are extremely rare and
metastases are far more common (see Fig. 85.43).116 Focal
or generalized pericardial thickening, enhancement,
nodularity, and hemorrhagic pericardial effusions are
some of the features that may be seen on CT. The common
primary malignancies that metastasize to the pericardium
are lung, breast, melanoma, and lymphoma.117
2066
CONCLUSION
MDCT has evolved rapidly over the last decade into a
powerful cardiac imaging tool that allows the physician to
visualize the coronary artery anatomy along with cardiac
morphology and function in a single noninvasive study.
Advances in technology now enable us to scan patients
with higher heart rates, decreased radiation dose, and
ability to overcome calcium artifacts. CTA compares
well to catheter angiography in detecting stenosis and,
in addition is superior in detecting regional wall-motion
abnormalities. Myocardial perfusion information can be
derived from rest and stress MDCT, although its wider
practical application is limited at this time. CT-FFR is
in early investigational state but could become a game
changer once it can be performed easily and quickly.
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multidetector computed tomography versus echocardiography for assessing regional left ventricular function. Am J
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2068
2069
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101. Siegel MJ, Bhalla S, Gutierrez FR, et al. MDCT of postoperative anatomy and complications in adults with
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102. Acharya D, Singh S, Tallaj JA, et al. Use of gated cardiac
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assessment of left ventricular assist devices with cardiovascular computed tomography and impact on
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2009;137(5):121317.
105. Little WC, Freeman GL. Pericardial disease. Circulation.
2006;113(12):162232.
106. Bull RK, Edwards PD, Dixon AK. CT dimensions of the
normal pericardium. Br J Radiol. 1998;71(849):9235.
107. Sechtem U, Tscholakoff D, Higgins CB. MRI of the
abnormal pericardium. AJR Am J Roentgenol. 1986;147(2):
24552.
108. Hynes JK, Tajik AJ, Osborn MJ, et al. Two-dimensional
echocardiographic diagnosis of pericardial cyst. Mayo Clin
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109. Wang ZJ, Reddy GP, Gotway MB, et al. CT and MR imaging
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110. Nasser WK. Congenital diseases of the pericardium. Cardiovasc Clin. 1976;7(3):27186.
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of Cardiovascular Medicine. 8th ed. Philadelphia, PA: WB
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Roentgenol. 2007;189(6):W31214.
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applications. Postgrad Med J. 2010;86(1013):16573.
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pericardium: appearances on ECG-gated 64-detector row
cardiac computed tomography. Br J Radiol. 2010;83(987):
194205.
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117. Klatt EC, Heitz DR. Cardiac metastases. Cancer. 1990;65(6):
14569.
Index
Page number followed by f and t indicate figure and table respectively.
A
Abdominal aortic aneurysm, ultrasound
stethoscope for screening of, 294
295
Aberration, 81
Abiomed Impella, 1224f
Abnormal mitral arcade, 16131614
Absorption, 5657
ACC. See American College of Cardiology
(ACC)
Accessory mitral orifice, 1615
ACCF. See American College of
Cardiology
Foundation (ACCF)
Accreditation Council of Graduate
Medical Education (ACGME), 754
Acetylcholine (Ach), 450, 451452, 451f,
455
Ach. See Acetylcholine (Ach)
ACM. See Alcohol-induced
cardiomyopathy (ACM)
Acoustic energy safety, 111
Acoustic enhancement, 734, 734f
Acoustic radiation force impulses (ARFI),
1995
Acoustic shadowing, 61, 62f, 733734
Acquired heart diseases, in childhood,
18561864
aortic insufficiency, 1860f
complications of, 1859
coronary ectasia and aneurysms,
18611863
Duke criteria for, 1857
echocardiographic findings, 1857
1859
infective endocarditis, 18561857
infective endocarditis prophylaxis,
1859
Jones criteria for, 18591861
acute valvulitis, 1860
chronic RHD, 18601861
minor criteria, 1860
Kawasaki disease, 1861, 1862t
mitral valve insufficiency, 1860f
overview, 1856
rheumatic heart disease, 1859
vegetations in, 18581859
location for, 1958
size and embolic risk, 19581959
ACS. See Acute coronary syndromes
(ACS)
AcuNav Diagnostic Ultrasound
Catheter, 644, 645f. See also
Intracardiac echocardiography
(ICE)
Acute aortic regurgitation, 1972
acute decompensation, 1974
acute regurgitation, 1974
aortic dissection, 1972
diastolic fluttering, 1974
infective endocarditis, 1972
left ventricular hypertrophy, 1974
post trauma, 1972t
suprasternal notch, 1974
transthoracic views, 1974
ventricular septal defect, 1973f
Acute chest pain, CT scan for, 20452047
Acute coronary syndromes (ACS)
contrast echocardiography for, 443
echocardiography role in, 12921294
evaluation of, by CONTISCAN
transducer, 226
Acute heart failure syndrome, 1986
Acute LV remodeling (ALVRM), 1325
Acute myocardial infarction, three
dimensional speckle tracking and,
376t
Acute pericarditis, 1436
Acute respiratory distress syndrome
(ARDS), 1983, 1986
acute heart failure syndrome, 1986
chest X-ray, 1986
differential diagnosis, 1986
diffuse lung injury, 1986
lung ultrasound scan, 1986
sonographic pattern
multiple diffuse B-lines, 1986, 1986f
small subpleural consolidations,
1986, 1986f
spared areas, 1986
subclinical pulmonary edema, 1986
Acute rheumatic fever (ARF), 931
diagnosis of carditis in, 765775
echo and Doppler studies, 768770
echocardiography vs. clinical
examination, 767768
echo interrogation, 767, 769t770t
M-mode echo in, 770
myocarditis, 766
pericarditis, 766
pitfalls in Jones criteria, 766767
regurgitation, 766
rheumatic vs. nonrheumatic
regurgitation, 768
secondary prophylaxis, fidelity of, 775
two-dimensional echo, 770772
overview, 765
physiological vs pathological
regurgitation, 772773
Vijaya's echo criteria, 771, 771t
Adenosine stress test, 13331334
ADMA (asymmetrical dimethyl arginine),
454
Adriamycin-induced cardiomyopathy,
1397f
Adult congenital heart disease (ACHD),
17911855
aortic root in, 1797, 1797f
complex congenital heart defects,
18261848
echocardiography, key concepts of,
17931797
abdominal situs, 1795
assessment of shunts, 17951796
atrial situs solitus, 1794
atrioventricular relationship, 1795
position of apex, 1794
pregnancy and, 17961797
simplified segmental approach,
17931795
VA connections, 1795
indications for TEE, 1792t
overview of, 17911793
I-II
simple congenital heart defects,
17981813
surgical terms in, 1794t
terminology in, 1793t
valvular disease, 18131826
Aging, effect of
on HV Doppler, 303
on pulmonary venous flow, 329330,
330f
Air embolism, 1977
coronary arteries, 1977
hemodynamic instability, 1977
paradoxical emboli, 1977
postcardiac arrest, 1977
post multiple injuries, 1977
respiratory distress, 1977
Air microbubble contrast, 436
AIUM. See American Institute of
Ultrasound in Medicine (AIUM)
ALa. See Anterior leaflet angle (ALa)
Alcohol-induced cardiomyopathy (ACM),
1397
Alcohol-induced necrosis, 1364f
Alcohol septal ablation
in HCM, 13621363
for hypertrophic obstructive
cardiomyopathy, 571
Alfieri stitch, 539
Aliasing, 70, 137, 736. See also Artifacts
Alveolar-interstitial syndrome, 1986
ALVRM. See Acute LV remodeling
(ALVRM)
Ambulatory echocardiography, 227229,
229t
American College of Cardiology (ACC),
750, 751752, 757, 758
American College of Cardiology
Foundation (ACCF), 758, 2045
American correction, 580
American Heart Association (AHA), 6, 758
American Institute of Ultrasound in
Medicine (AIUM), 110
American Registry of Diagnostic Medical
Sonography (ARDMS), 754
American Society of Anesthesiologists
(ASA), 638
American Society of Echocardiography,
907
American Society of Echocardiography
(ASE), 91, 132, 296, 516, 638, 753,
1115, 1323
A-mode echocardiography, 57, 58f
Amplatzer PFO Occluder, 557
AmplatzerTM atrial septal occluder,
552553, 554f
Index
acute aortic diseases, 976982
aortic aneurysm, 974976
aortography, 980
atherosclerotic disease, 971974
celiac artery, 970
descending thoracic aorta, 970
human anatomic cross-section, 968f
intramural hematoma, 978
left carotid and subclavian artery,
970, 971f
magnetic resonance imaging and,
979
orientation, 968970
real time 3D, 981
transgastric level, 969
Aortic abscess
in native valve, 1045f
transesophageal approach, 1053f
Aortic aneurysms, 951954, 16951696,
1797
anomalous origin of PA from
ascending aorta, 16951696,
1696f
echocardiographic evaluation of,
951954, 952f954f
in elderly, 19341937
cystic medial degeneration and, 1936
etiology of, 1936
natural history of, 1937
with rupture, 1936f
epidemiology of, 951
pathophysiology of, 951
pulmonary artery sling, 1696
transesophageal imaging of, 974976
Aortic arch (AoA), 664665, 665f, 666f,
1538f, 1795f
abnormities, 16901692
cervical aortic arch, 1691
right aortic arch, 16901691
vascular rings, 1691
anomalies, 665
coarctation of aorta, 16921694
interruption of, 16941695, 1695t
types of, 664665, 665f
Aortic arch anomalies, 17701773
aortic arch to pulmonary artery
fistula, 1773
coarctation of aorta, 17701773
Aortic arch scanning protocol, carotid
ultrasound, 676f
Aortic atheroma, 959961
appearance on TTE and TEE, 959,
960f
grading, 961t
penetrating aortic ulceration, 961,
962f
TEE characterization of, 960961
Aortic atherosclerosis
in elderly, 19211923, 1922f
epiaortic ultrasonography in,
640641, 641f
in female, 1896f
Aortic balloon valvuloplasty (ABV), 541
Aortic coarctation. See Coarctation of
aorta (CoA)
Aortic dissection, 954958, 1974
aorta distal to left subclavian artery,
954
in ascending aorta, 954
diagnostic imaging, 1974
dissection, 1974
in elderly, 19371942, 1937f1938f,
1941f
acute coronary syndrome and, 1940
color Doppler flow in, 1942
descending thoracic aorta, 1942
perfusing lumen, 1942
rupture, 1938f1940f
epidemiology, 954
intramural hematoma, 957958
TEE diagnosis of, 955957
TTE evaluation, 955
Type A dissection, 954, 955957
Type B dissection, 954
Aortic dissection, epiaortic
ultrasonography for detection of,
641
Aortic insufficiency, 1860f
and carotid ultrasound findings, 693
Aortic isthmus, 1538f
Aortic leiomyosarcoma, 1489f1490f
Aortic lumen to pulmonary artery fistulas
(APAF), 1773
Aortico-left ventricular tunnel, 1632
Aortico-right atrial tunnel, 1632
Aortico-right ventricle tunnel, 1632
Aortic override, 16331644
aortic arch, 16381639
aortopulmonary collaterals, 1639
1640
coronary artery anomalies, 1640
patent arterial duct, 16391640
pulmonary arteries, 16371638, 1638f
pulmonary stenosis, 16341637
tetralogy of Fallot with absent
pulmonary valve, 1637, 1637f
cardiac catheterization, indications
for, 1641
echocardiographic measurements in,
16401641, 1640f
postoperative evaluation of, 1641
1644, 1642f
ventricular septal defect, 16331634,
1633f1634f
Aortic regurgitation (AR), 806812,
930944, 16281630, 1630f
in adults, 1824
aortic root dilatation, 16291630
aortic valve surgery, timing of,
941942
cardiac MRI for, 20102011, 2011f
causes of, 16281629, 1629t
continuous wave Doppler, 810811
signal of, 1279
3DE VCA measurement of, 280
diastolic fluttering of AML, 807808
echocardiography
exercise, 811
indications for, 807t
role of transthoracic, 811
three-dimensional, 812
etiology of, 930935
acute rheumatic fever, 931
aortic root and aortic annulus,
diseases of, 935, 936f
aortic valve cusps, diseases of,
930935, 932f935f
Behet's disease, 935
congenital cardiac defects and aorta
abnormalities, 930931
infective endocarditis, 931
systemic illnesses, 931
flow convergence, 809
follow-up in, 812
grading of, 810f
left ventricle adaptation to, 941
left ventricular outflow abnormality,
16281629
pulsed wave Doppler, 810
regurgitant jet size, 808809
severity, quantification of, 936941
color Doppler methods, 936939
continuous wave Doppler methods,
940941, 941f
mitral valve findings, 941
M-mode and 2D findings, 936, 937f
parameters to grade, 937t
pulsed wave Doppler methods,
939940, 940f
severity of, 808, 808t, 812, 1630
sinus of Valsalva aneurysm and, 1631
vena contracta imaging in, 938
Aortic root, 1621
I-III
I-IV
etiology of, 897898
low-gradient, low stroke volume,
severe, 908911
aortic valve calcium score, 911
dobutamine infusion, 910
hypertension and, 911
low transvalvular gradients and, 913
mean transaortic pressure gradient,
804805
normal aortic valve anatomy, 896897
aortic root, 897
aortic valve, 896897
oveview, 896
pseudosevere group of, 911
sclerosis to stenosis, 897898
severity, estimation of, 803, 907908,
907t
definition of, 907, 907t
methodological limitations in, 908,
908t, 909t
pitfalls in estimation of, 908
technical errors in, 908
strain in evaluation of, 913
subvalvular, 16241626
supravalvular, 16261628
valve area, 805
valvular, 16181624
vs. hypoplastic left ventricle, 1623
Aortic trauma, and free rupture, 961963
Aortic valve (AV), 582584
anatomy of, 896897
3DE assessment of, 278f, 279, 279f
3D echo of, 520522, 520f
intermittent opening of, 1242f
short axis of, 1536f
ventricular assist devices, 1231, 1231f
Aortic valve, in adults, 18211826
aortic regurgitation, 1824
aortic stenosis, 1824
bicuspid, 18211824
cardiac catheterization, 18221823
echocardiography, 18211822
MRI/CT for, 1823
postoperative adult, 1824
stress testing, 18231824
subaortic stenosis, 18241825
supravalvular aortic stenosis,
18251826
Aortic valve annulus, 1621
Aortic valve area (AVA), 279, 1622
calculation of, 543, 585586
Aortic valve calcium score:, 911
Aortic valve diseases, 802816, 1318
aortic regurgitation, 806812
continuous wave Doppler, 810811
3D echocardiography, 812
diastolic fluttering of AML, 807808
exercise echocardiography, 811
flow convergence, 809
follow-up in, 812
grading of, 810f
pulsed wave Doppler, 810
regurgitant jet size, 808809
role of TEE in, 811
severity of, 808, 808t, 812
aortic stenosis, 802806
aortic jet velocity, 803804
associated conditions, 806
3D echocardiography, 806
mean transaortic pressure gradient,
804805
severity of, 803
stress echocardiography in, 805806
valve area, 805
role of 3D TEE in operating room in,
582588, 594f600f
stress echocardiography in, 1318
Aortic valve Doppler examination,
904912
Aortic valve fibroelastoma, 1479f
Aortic valve pseudoaneurysm, 1046f
Aortic valve sclerosis, 1923f
in elderly, 19231924, 1923f
bicuspid, 1931f
echocardiographic findings for,
19231924, 1930f
normal hemodynamics in, 1924
pathophysiology of, 19231924
prevalence of, 19231924
systolic murmur, 1923
Aortic valve stenosis (AS)
in adults, 919
calcific, 919
dobutamine stress echocardiography,
response to, 921
invasive evaluation of, 923
invasive vs. noninvasive evaluation
of, 922924
SAVR role in, 926927
severity grade, 919
three main entities of severe, 926t
Aortocameral communications, 1632
aortico-left ventricular tunnel and,
1632
aortico-right atrial tunnel and, 1632
aortico-right ventricle tunnel and,
1632
Aortopulmonary window (APW), 1751,
1753f1754f
Index
color flow mapping, 1603
types of, 1602
APAF. See Aortic lumen to pulmonary
artery fistulas (APAF)
Aperture
3D ultrasound imaging and, 80
and TEE image quality, 104, 106f
Apical approach, of three-dimensional
echocardiography, 244, 254f, 255f
Apical hypertrophic cardiomyopathy,
1356
Apical window, TTE, 146, 148
five-chamber plane, 153154, 153f,
154f, 154t
Doppler imaging, 153154, 154f, 154t
technique, 153, 153f
four-chamber plane, 148153,
148f153f, 149t
chamber quantification, 150151,
151f, 152f
diastolic function assessment,
149150, 150ff, 151f
Doppler imaging, 149, 150f
intracardiac shunts assessment, 153,
153f
right heart assessment, 151152, 152f
technique, 148, 148f, 149t
two-dimensional anatomic imaging,
148149, 149f
long-axis or three-chamber plane,
155, 156f
two-chamber plane, 154155, 154f,
155f
chamber quantification, 154155
Doppler imaging, 155
technique, 154, 154f
two-dimensional anatomic imaging,
154, 154f, 155f
Application specific integrated circuits
(ASICs), 77, 77f, 78
ARDMS. See American Registry of
Diagnostic Medical Sonography
(ARDMS)
Area under the curve (AUC), 452, 452f
ARFI. See Acoustic radiation force
impulses (ARFI)
ARIC. See Atherosclerotic Risk in
Communities (ARIC) study
Arrhythmogenic right ventricular
cardiomyopathy (ARVC), 1395
1396
Arrhythmogenic right ventricular
dysplasia (ARVD), 395396, 1395
1396, 1396f
radiofrequency catheter ablation,
2056
and HV Doppler, 309, 309f
and PV Doppler, 333, 333f
TD imaging in, 353
Atrial flutter, and PV Doppler, 333, 333f
Atrial myxoma, 1468
Atrial septal defects (ASD), 1134, 1279,
15851591, 1734
in adults, 17991802, 1801t
cardiac catheterization, 1802
closure of, 1802
contrast echocardiography, 1801
1802
echocardiography, 1799
exercise testing, 1802
MRI/CTA for, 1802
postoperative adult in, 1802
transesophageal echocardiography,
18001801, 1800f
types of, 17991800, 1799f
atrial septum, evaluation of, 1590
basal long-axis or bicaval view,
15901591
basal short-axis view, 1590
four-chamber view, 1591
cardiac catheterization in, 1590
closure of
Amplatzer ASD occluder, 557f
ASD closure using intracardiac
echocardiography, 559f
ASD sizing ballon, 556f
closure devices, 552553, 555f
3D TEE diagnosis of ASD, 551, 553,
553f
3D TEE monitoring of, 554555, 557f,
558f
3D TEE sizing of ASDs, 556f
3D TEE visualization of ASD rim, 557f
ICE imaging during, 648, 650, 650f
indications for, 551
secundum ASDs, 552, 554f
computed tomography for, 2059,
2060f
coronary sinus, 1586
degree of left-to-right shunt in, 1590
direction of shunt, 15881589
3D TEE for, 513
echocardiographic imaging in,
15861588
fossa ovalis, 1586
for percutaneous device closure, 1591
objectives, 1585
ostium primum, 1586
patent foramen ovale, 15851586
I-V
and PV Doppler, 342, 342f
Qp/Qs ratios in, 15891590
secundum, 17341742
sinus venosus, 1586, 17421743
stepwise evaluation for, 1586t
three-dimensional speckle tracking
and, 376t
types of, 15851586
in women, 19001901, 1900f1901f
Atrial septum, 1248, 1962
ablation,patients, 1962
abnormalities, 1962
frequent, 1963
atrial tumor, 1963
fossa ovalis region, 1962
lipomatous, 1963
obesity, patients, 1963
saline contrast study, 1962
septum primum, 1962
septum secundum, 1962
trans-septal puncture, 1962
Waterstons groove, 1963
Atrioventricular and ventricoarterial
discordance, 16641670
abnormalities of RVOT, 1669
associated defects, 1664
associated malformations, 16671669
tricuspid value abnormalities,
16671669, 1668f
ventricular septal defect, 1667, 1668f
coarctation of aorta, 1669
coronary artery anatomy in, 1664
1667, 1665f1666f
four-chamber views, 16651666
PLAX view, 1667
segmental analysis, 16641665
subcostal sagittal view, 16661667
left ventricular outflow tract
obstruction, 1669
Atrioventricular canal defect (AVCD),
1535, 1544f, 1546f
Atrioventricular connection,
echocardiography of, 1558t,
15771579, 1578f
concordant, 1579
discordant, 1579
isomeric, 1580
and loop rule, 1580
uniatrial and biventricular
connection, 1580
univentricular, 15791580
Atrioventricular dissociation
and HV Doppler, 308, 308f
and PV Doppler, 331332, 332f
I-VI
B
Balloon mitral valvotomy, in mitral
stenosis, 839t, 842f
complications of, 840841
echocardiography in patients for,
838840
evaluation of patient, 838840, 839t
mitral regurgitation in, 840841
post, 841f, 843f, 844f
Balloon valvuloplasty, monitoring of, by
CONTISCAN transducer, 233, 233f,
234f
Barlows syndrome, 1860
Index
C
Calcific aortic stenosis. See also Aortic
stenosis
2D echocardiography in, 900
effective orifice area in, 900
geometric orifice area in, 900
Calcium channel blockers (CCB), 1073t
Calcium-related factors, in cardiac
motion, 1202
Cannon wave, 308, 308f
Cannula flow velocities, 1248
Capacitance micro-machined ultrasound
transducer (cMUT), 1992
Capacitive micromachined ultrasonic
transducer (CMUT), 115f, 116, 116f
Capillary blood volume (CBV), 421422
Capillary wedge pressure (CWP), 1063
Carcinoid heart disease, 1006, 14071413,
1500
with carcinoid syndrome, 1408
cardiac involvement in, 1408
2D transthoracic echocardiography,
1007f
echocardiographic features, 1409
1413
endocardial deposits, 1409,
1410f1412f
left-sided valvular involvement, 1409,
1410f
pulmonary valve cusps, 1409
tricuspid regurgitation, 1412
Carcinoid tumors, 18741875, 1876f
Cardiac amyloidosis, 352, 373, 1261
Cardiac MRI for, 2008f
Cardiac calcified amorphous tumor
(CAT), 15121514
Cardiac catheterization
in atrial septal defects, 1590
of bicuspid aortic valve in adults,
18221823
in CCTGA, 1841
in coarctation of aorta, 1829
double outlet right ventricle, 1845
in D-transposition of great arteries,
1838
echo gradients during, 1574, 1575f
indications for, tetralogy of Fallot and,
1641
truncus arteriosus, 1844
univentricular hearts, 18471848
Cardiac computed tomography, 2023
2070
advantages of, 2024
anomalous pulmonary venous
connection, 2059
aorta congenital heart disease,
coarctation of, 2062, 2063f
atrial septal defect, 2059, 2060f
cardiac function, 2042, 2043f
CCTA, contraindications for, 2027
challenges for, 20242025
cardiac gating, 20242025
spatial resolution, 2024, 2024t
temporal resolution, 2024, 2024t
clinical indications for, 20442066
acute chest pain, 20452047
anomalous coronary artery, 2047
2049, 2047f
asymptomatic intermediate risk
patient, 20492050
cardiac masses, 20512054
congenital heart disease, 20592063
coronary artery bypass graft, 2050,
2052f
coronary calcium scoring, 20442045,
2045f, 2046f
coronary stent evaluation, 2050
left ventricular assist device, 2063
2064
pericardial diseases, 20642066
pulmonary vein ablation, 20542057
re-do coronary artery bypass graft,
2049
constrictive pericarditis, 2065
contrast media and injection and,
2029t
coronary calcium score protocol,
2028t
FOV for, 2027
image analysis, 20322034
extracardiac findings in, 20332034,
2037f
proper phase selection, 2033
image postprocessing, 20282032
curved multiplanar reformation, 2029
maximum-intensity projection, 2029,
2032f
multiplanar reconstruction, 2029,
2301f
transaxial images, 20282029
virtual endoscopy, 2030
volume rendering, 2030
major adverse cardiovascular events
in, 2042
in myocardial perfusion, 20422043
overview, 20232024
patient preparation for, 2028t
patient selection for, 2027
pericardial calcification, 2065, 2066f
pericardial defects, 20642065
pericardial duplication cyst, 2064
pericardial effusion, 2065, 2065f
pericardial tumors, 20652066
pitfalls and artifacts, 20342040
beam-hardening artifact, 2038, 2040f
blooming artifacts, 20362038, 2039f
cardiac motion artifact, 20342036
misregistration artifact, 2038f
respiration motion artifact, 2036,
2039f
plaque characterization, 20412042,
2041f
preablation pulmonary valve
mapping, 2028t
radiation dose, 20252027
of common examinations, 2026t
descriptors, 2026t
methods to reduce, 20262027, 2027t
I-VII
stress myocardial imaging using,
20422043
technique, 2027, 2028t, 2029t
tetralogy of fallot, 2062, 2063f
transcatheter aortic valve
replacement, 2029t, 20572059
transposition of great arteries,
20592062, 2061f
congenitally corrected, 2062, 2062f
Cardiac contusion, 1971
biochemical cardiac markers, 1971
echocardiogram, 1971
echo findings sum, 1971
electrocardiogram, 1971
myocardium, 1971
post trauma, 1972
Cardiac fibroma, 2054f
Cardiac hemangiomas, 14821484
Cardiac hypertrophy, cardiac MRI in,
20062008
Cardiac imaging, 34. See also
Echocardiography
Cardiac implantable electronic devices
(CIED), 1967
cardiac arrest, 1967
cardiac resynchronization therapy,
1967
echocardiography, 1967
infective endocarditis, 1967
heart disease, 1967
coronary artery disease, 1967
dilated cardiomyopathy, 1967
hypertrophic cardiomyopathy, 1967
lead extraction, 1967
preprocedural selection, 1967
symptomatic bradycardia, pacing,
1967
ventricular arrhythmias, 1967
Cardiac lipomas, 14811482, 1483f
Cardiac magnetic resonance imaging, in
ICM/NICM, 14271428
Cardiac masses, role of 3D TEE in
operating room in, 617627,
625f634f
Cardiac motion, 11761209
calcium-related factors in, 1202
clinical implications, 1201
definitions, 11831184
diastolic dysfunction, 12011202
dilated cardiomyopathy and,
12021203
heart function, basic, 11771180,
1177f1180f
MRI phase contrast velocity mapping
of, 1179f
I-VIII
myocardial fiber organization, 1180f
time frames of systole and diastole,
1177f1178f
during isovolumic contraction, 1185f
myocyte factors in, 1202
of normal heart, 11851194
overview, 11761177
pacing factors in, 12031204
right heart failure and, 1204
right ventricle, 1198
rotation, 1183
state-of-the-art, 11801181
composite of, 11811183
HVMB model, 1181
muscle contraction, asynchronous,
1181
subendocardial muscle, 11981200,
1199f, 1200f
torsion, 11831184
twisting, 1183
untwisting, 1183
mitral valve opening and, 12001201
torsion and, 11841185, 1200
ventricular septum, 11941198
ventricular structure, function of,
1177f1178f
Cardiac MRI (CMR), 381
cardiac amyloidosis, 2008f
for cardiac hypertrophy, 20062008,
2007f
cardiomyopathies, 2008
for left and right ventricle, 19981999,
1999f
for left ventricular structure, 2000
2004
LVNC, 20002002, 2001f, 2002f
tissue characterization, 20022003,
2003t
limitations of, 20172019
for mitral valve, 2012
for myocardial infarction, 20032004,
2004f
for myocarditis and sarcoidosis,
20042006, 2005f, 2006f
for normal variants and masses,
20162017
overview, 1998
pericardial disease, 20142016, 2015f
of prosthetic valves, 20132014
strain assessment, 19992000, 2000f
for valvular heart disease, 20092013
aortic regurgitation, 20102011, 2011f
aortic stenosis, 2010, 2011f
mitral regurgitation, 20122013
mitral stenosis, 2013
tricuspid regurgitation, 2013, 2014f
velocity mapping, flow and shunt
assessment, 20082009
viability assessment, 20032004
Cardiac myxoma, 14641475
Cardiac output (CO), 230, 231f, 1280, 1281
Cardiac Performance Analysis (CPA), 380
Cardiac plasmacytoma, 14901491, 1492f
Cardiac resynchronization therapy (CRT),
233234, 365, 1372
for heart failure, 369, 370f
LBBB and, 1377
Cardiac rhabdomyoma, 14801481
Cardiac sarcoidosis, 3D speckle tracking
and, 376t
Cardiac shunts, 171
Cardiac situs, 1531f
Cardiac tamponade, 14381440, 1970
blunt force, 1970
cardiac involvement, 1970
clinical presentation, 1970
and HV Doppler, 317, 317f
left ventricular outflow tract
diameter, 1974
myocardial contusion, 1970
pericardial effusion, 1970
pulsed wave Doppler, 1443f
and PV Doppler, 341
right heart chamber collapses in,
1442t
right ventricular wall, 1971t
transmitral inflow velocities, 1970
transtricuspid velocities, 1970
tricuspid valve, 1971t
Cardiac transplantation
2D STE and, 371
velocity vector imaging in, 399400,
399f
Cardiac tumors and masses, 14621523
atrial thrombus, 1504
cardiac thrombi, 1504
cardiac vegetation, 15061511
Chiari network, 1503
computed tomography for, 20512054
Coumadin ridge, 15031504
crista terminalis, 1503
differential diagnosis of, 14621463
echocardiographic assessment of,
14621464
3D TTE, 1464
full-volume acquisitions, 1463
limitation of, 1463
papillary fibroelastomas, 1463,
1476f1477f
Eustachian valve, 1503
Index
left ventricular thrombus, 15041506
malignant primary cardiac tumors,
14841511
angiosarcoma, 14851486
cardiac plasmacytoma, 14901491,
1492f
fibrosarcomas, 1488
hydatid cyst, 1491, 1493f1498f
pericardial mesotheliomas, 1491
primary cardiac lymphoma, 1490
rhabdomyosarcoma, 14861488
sarcomas, 14841488
mesothelial/macrophage incidental
cardiac excrescences, 1511
1518
cardiac calcified amorphous tumor,
15121514
extracardiac masses, 1514, 1514f
intracardiac hardware, 15141517
mitral annular calcification, 1512,
1512f
moderator band, 1503
perivalvular abscess, 1511
primary benign cardiac tumors,
14641484
benign cardiac fibromas, 1480
cardiac hemangiomas, 14821484
cardiac lipomas, 14811482, 1483f
cardiac myxoma, 14641475
cardiac rhabdomyoma, 14801481
papillary fibroelastomas, 14751480
secondary cardiac tumors, 14921500
carcinoid heart disease, 1500
malignant melanoma, 1500
thebesian valve, 1503
Cardiomyopathies. See also specific
Cardiomyopathy
diabetic, 1407, 1408
dilated. See Dilated cardiomyopathy
(DCM)
2D STE and, 369371
hemochromatosis, 1405
infectious, 14051407
HIV-associated cardiomyopathy,
1407
septic cardiomyopathy, 14051407
infiltrative, 1405
left ventricular noncompaction,
13841388
echocardiographic features of,
13851388, 1386f1387f
isolated, 1387f
normal fetal ontogenesis, 1385
metabolic, 1407
in neuromuscular disorders, 396
peripartum, 13811384, 1381f1384f
primary, 1370
sarcoidosis, 1405, 1405f
secondary, 1370
tachycardia-induced, 1388, 1395f
Takotsubo cardiomyopathy, 1388,
1394f
toxic, 13961397
adriamycin-induced
cardiomyopathy, 1397f
alcohol-induced cardiomyopathy,
1397
chemotherapy-induced
cardiomyopathy, 13961397
two-dimensional echo, 1407
Cardiopulmonary bypass, and HV
Doppler, 317, 318f
Cardiopulmonary ultrasound, 1987
acute lung injury/ARDS, 1987
B-lines, 1987
evaluation, 1987
cardiogenic pulmonary congestion,
1987
echocardiography, 1987
echography, 1987
lung ultrasound scan, 1987
addition of, 1987
findings, 1987
helps in, 1987
pathological conditions, 1987
pulmonary fibrosis, 1987
three B-line scenarios, 1987, 1987t
valvular heart disease, 1987
Cardiothoracic ratio, 1531f
Cardiovascular Credentialing
International (CCI), 754
Carditis, diagnosis of, 765775
autopsy, 771f
echo and Doppler studies, 768770
echocardiography
superiority of, 773774
two-dimensional, 770772
vs. clinical examination, 767768
echo interrogation, 767, 769t770t
M-mode in, 770
myocarditis, 766
pericarditis, 766
pitfalls in Jones criteria, 766767
regurgitation, 766
rheumatic vs. nonrheumatic
regurgitation, 768
secondary prophylaxis, fidelity of, 775
Carotid artery, 673f
Carotid bulb, 667, 668f
I-IX
I-X
postoperative adult, 1829
stress echocardiography, 1828
surgical intervention for, 1829
transesophageal echocardiography,
18271828
treadmill stress testing, 1828
double outlet right ventricle, 1824,
1825
D-transposition of great arteries,
18351840, 1837t
cardiac catheterization, 1838
echocardiography, 18361838
MRI/CT for, 1838
postarterial switch repair, 1838
postatrial switch repair, 1836
reoperation for, 18381840
stress echocardiography, 1838
tetralogy of Fallot, 18291835, 1833t
cardiac catheterization, 18341835
echocardiography, 18301832
MRI/CT for, 1835
postoperative adult, 1835
postoperative adult, surgery in, 1835
stress echocardiography, 18321834
truncus arteriosus, 18421844, 1843t
cardiac catheterization, 1844
echocardiography, 1843
MRI/CT for, 1844
postoperative adult, 1844
surgery for, 1844
univentricular hearts, 18451848
cardiac catheterization, 18471848
echocardiography, 18461847
MRI /CTA for, 1847
transesophageal echocardiography,
1847
tricupsid atresia and post-fontan
adult, 1845, 1846f
Computational fluid mechanics, 67
Computed tomography angiogram (CTA)
flow limiting disease, accuracy in,
2044
preoperative, 2049, 2050f
retrospective gated, 2043f
Congenital anomalies
of coronary arteries, 13431344
of mitral valve, 16101615
individual mitral lesions, evaluation
of, 16111615
mitral valve lesions,
echocardiographic views,
16101611
types of, 1611t
of tricuspid valve, 16161618
Index
congenitally unguarded
tricuspid orifice, 16171618, 1617f
Ebsteins anomaly of, 16161617,
1616f
tricuspid valve prolapse, 1617
Congenital aortic stenosis, 17661770
Congenital bicuspid aortic valve,
16191620, 1619f
Congenital complete heart block (CCHB)
fetal, 1529, 1545
Congenital cystic adenomatoid
malformation (CCAM), 1530
Congenital diaphragmatic hernia (CDH),
1530
Congenital disorders of glycosylation
(CDG), 1407
Congenital double orifice mitral valve, in
mitral stenosis, 844845
Congenital heart defects, simple, in
adults, 17981813
shunt lesions, 17981813
atrial septal defects, 17991802, 1801t
atrioventricular septal defect,
18091811
coronary artery fistula, 18121813
patent ductus arteriosus, 18051809
patent foramen ovale, 17981799
persistent left superior vena cava,
18101811
sinus of Valsalva aneurysm, 1811
1812
ventricular septal defects, 18021805
Congenital heart disease (CHD), 1733
1790, 19001902
aortic arch anomalies, 17701773
aortic arch to pulmonary artery
fistula, 1773
coarctation of aorta, 17701773
aortopulmonary window, 1751
atrial and atrioventricular valve
abnormalities, 17731776
cor triatriatum sinister, 17731775
Ebsteins anomaly, 17751776
isolated mitral valve cleft, 1775
atrial septal defects, 19001901
Chiari network, 17761779
common atrium, 17471751,
1751f1752f
computed tomography for, 20592063
conotruncal anomalies, 17541766
anomalous coronary artery, 1763
1766
tetralogy of fallot, 1763
transposition of great arteries,
17541763
dextrocardia, 15701575, 1570f
Bernoulli equation, limitations of,
1574
color flow Doppler, 15701572
color flow information, 1572
continuous wave Doppler, 1572
Doppler gain and filter settings,
inappropriate, 1571f, 1572f,
1573
Doppler scales, inappropriate, 1573,
1573f
echo gradients during cardiac
catheterization, 1574, 1575f
nonimaging continuous wave
Doppler probe, pitfalls of, 1573
poor echo windows, 1572
pulsed Doppler, 1572
signal alignment, inappropriate,
1573, 1574f
transducer frequency, inappropriate,
1573
trivial lesions, pressure gradients
across, 1574
double outlet right ventricle, 1779
1783
left ventricular-RA communication,
17791782
right coronary artery fistula, 1782
1783
echocardiography (imaging) of,
15631570
apical view, 15661567, 1566f
conventional views of, 1563t
high parasternal or ductal view, 1569,
1569f
long-axis view, 1569, 1569f
parasternal long-axis view, 1567,
1567f
parasternal short-axis view, 1568
1569, 1568f, 1569f
pitfalls in, 15711572, 1571t
short-axis view, 1570, 1570f
subcostal window, 15631566,
1564f1565f
suprasternal views, 1569, 1569f, 1570f
and HV Doppler, 317318, 318f
other abnormalities, 17761779
outflow tract obstruction, 17661770
congenital aortic stenosis/bicuspid
aortic valve, 17661770
subaortic stenosis, 1770
overview, 1733
patent ductus arteriosus, 17511754,
1755f1757f
patient preparation, 15621563, 1562f
pulmonary hypertension, 19011902
RT 3DE approach to, 1170
sequential chamber analysis,
principle of, 15751582
atrial arrangement, identification of,
15751577
atrioventricular connection, 1577
1579
ventricular arterial connection,
15791582
shunt lesions/septal defects, 1733
1747
atrial septal defects, 1734
atrioventricular septal defectsts
17461747
secundum ASD, 17341742
sinus venosus ASD, 17421743
unroofed coronary sinus, 1743
ventricular septal defects, 17431746
sinus of Valsalva aneurysm, 1784
hypoplastic left heart syndrome, 1784
right ventricular outflow obstruction
and, 1784
Congenitally corrected transposition of
great arteries (CCTGA), 18401842
cardiac catheterization, 1841
echocardiography, 18401841
exercise testing with stress
echocardiography, 1841
magnetic resonance imaging, 1841
postoperative adult, 1842
surgery for, 18411842
velocity vector imaging in, 392
Congenitally corrected transposition of
great vessels (CTGA), 1664, 1664f
Congenitally stenotic tricuspid aortic
valve, 1620
Congenitally unguarded tricuspid orifice,
16171618, 1617f
Congenital mitral stenosis, 844
Congenital parachute mitral valve, 845,
846f
Congenital subaortic membrane, 1359
Conotruncal anomalies, 17541766
anomalous coronary artery, 1763
1766
tetralogy of fallot, 1763
transposition of great arteries,
17541763
Constrictive pericarditis (CP), 14441448
Doppler flow velocity records,
14461448, 1447f
3D TTE vs. 2D TTE, 14561457
I-XI
echocardiographic findings in, 1445t
effusive, 1448f
hepatic vein and, 1447f
and HV Doppler, 315317, 316f, 317f
M-mode and 2D echo, 1444, 1445f,
1446f
and PV Doppler, 340341, 340f, 341f
Continuity equation, 923
for aortic valve area, 906907
for MVOA, 789
Continuous infusions, 421
Continuous wave Doppler, 6364. See
also Spectral Doppler
disadvantage of, 6869
methodology, 6869, 69f
Continuous-wave jet intensity, MR and,
869
CONTISCAN transducer, 224237
use of, 224225, 225f
acute coronary syndromes,
evaluation of, 226
ambulatory echocardiography,
227229
balloon valvuloplasty, monitoring of,
233
cannulation of coronary sinus,
monitoring of, 233236
exercise echocardiography, 226227
intraoperative monitoring, 236237
noninvasive hemodynamic
monitoring, 229230
pericardiocentesis, monitoring of,
230233
Contractile reserve, assessment of, 276
Contrast CMR (gadolinium), 2020
Contrast defect area (CDA), 423
Contrast defect length (CDL), 423424
Contrast echocardiography, 416436,
737, 1308. See also Myocardial
contrast echocardiography (MCE)
contrast administration
bolus injection, 421
continuous infusion, 421
contrast agents, 416417, 417f
commercially available, 417, 418t
idle, properties of, 417
safety of, 434435, 435t
ultrasound and, interaction between,
418419, 419f
use of, 428431
contrast imaging, physics of, 417418,
418f
history of development of, 1113
imaging modalities, 420, 420f
I-XII
intermittent/triggered imaging,
420421
real time imaging, 420
indications for, 426
enhanced endocardial border
delineation (EBD), 426, 426f
left ventricular opacification (LVO),
426427, 427f429f
myocardial perfusion, evaluation of,
427428, 429f
myocardial perfusion, principles of
assessment of, 421422, 422f
contrast modality during MCE,
422423
qualitative MCE, 423, 424f, 425f
quantitative MCE, 424, 426
semiquantitative MCE, 423424, 425f
and problems, 431
attenuation artifacts, 431433, 432f,
433f
blooming, 433, 434f
swirling, 433, 434f
thoracic cage artifacts, 433434, 434f
saline, 435, 436f
training in, 757
Contrast microbubbles, 416417
Contrast score index, 441
COPD. See Chronic obstructive
pulmonary disease (COPD)
CoreValve, self-expandable, 541542, 543
Coronary aneurysms, 16881689
atresia of left main coronary artery,
1689
Kawasaki disease, 16881689
Coronary anomaly, 1344f
Coronary arteries, 13371347
anatomy and physiology, 1337
congenital abnormalities of, 1343
1344
coronary flow reserve, 13401343
coronary ostia, 1338
coronary stenosis, 1338
distal coronary flow, 13401343
normal left main, 1339f
overview, 1337
parasternal short-axis view for, 1569,
1569f
proximal, 1338f, 1339f, 1341f, 1341t
visualization of, 13371340
transesophageal echocardiography,
13381340
transthoracic echocardiography,
13371338
Coronary arteriovenous fistulas, 1688,
1689f
Index
D
Dark blood imaging (spin echo), 2020
Data acquisition methods, 3D
echocardiography
cropping, 269270, 269f
3DE color flow Doppler imaging, 269
image display, 269270
multiplane mode, 268269
multiple-beat 3DE imaging, 269
real time 3DE, 269
tomographic slices, 270, 270f
3D beamformer, 7778
DDD pacing, for obstructive HCM,
1363
Debakey classification, 1974
aortic arch, 1974
ascending aorta, 1974
proximally, 1974
Deceleration time (DT), 313
systolic function in, 1371
ventricular remodeling, 1379
wall motion abnormalities in,
13711372
Dilated pulmonary valve, 1555f
Dipyridamole stress test, 1334
Discrete subaortic membrane,
1771f1772f
Displacement encoding with stimulated
echoes (DENSE), 1144
Distal coronary flow, 13401343
D-malposition
of aorta, 1548f
of great arteries, 1548f
Dobutamine stress echocardiography
(DSE), 274, 1307
Dobutamine stress test, 13311333
Dominance, 1181
Doppler, 1993
across-line Doppler shifts, 1993
beam-to-vessel angle, 1993
color Doppler, 1993
postacquisition, 1993
Doppler, Christian, 9
Doppler angiography. See Power Doppler
Doppler equation, 65, 66
Doppler hemodynamics, with stress
echocardiography, 13181319
aortic valve disease, 1318
dynamic pulmonary hypertension,
13181319, 1319f
hypertrophic cardiomyopathy, 1318
latent diastolic dysfunction, 1318
mitral valve disease, 1318
Doppler principle, 349
Doppler shift, 65
Doppler spectral display, 6768
Doppler tissue imaging. See Tissue
Doppler
Doppler ultrasound, 911, 6364, 6573
color Doppler, 64, 71
continuous wave Doppler, 6364,
6869
Doppler velocity determination, 7273
history of development of, 911
information from, 73
instrumentation, 6668
physical principles, 63
power Doppler, 7172
pulsed wave Doppler, 6364, 6970
tissue Doppler, 64, 72
Doppler velocity determination, 7273
errors in, 72
DORV. See Double outlet right ventricle
(DORV)
I-XIII
I-XIV
cardiac catheterization, 1838
echocardiography, 18361838
MRI/CT for, 1838
postarterial switch repair, 1838
postatrial switch repair, 1836
stress echocardiography, 1838
velocity vector imaging in, 392
Dual plane transesophageal echo (TEE)
probe, 99100, 100f
Duchenne muscular dystrophy (DMD),
396
Ductal arch (DuAr), 1537f
Ductus arteriosus, 548
Ductus venosus (DV), 1532f, 1538f
Duplex scanning protocol, carotid
ultrasound, 672f
DuraHeart magnetically levitated
centrifugal assist system, 1228
Dynamic pulmonary hypertension, stress
echocardiography in, 13181319,
1319f
Dysplasia of mitral valve, 1613
Dysplastic pulmonary valve, 1554f
E
Early cardiac flow Doppler era, 2449
color Doppler, development of,
3738, 41f44f
diastology, 4748
directional Doppler flowmetry, 2930
Doppler presentation, controversy
on, 3031
flow concept, emergence of, 2728
heart cavities, exploring of, 33, 34f
intracardiac Doppler flow velocity
traces changes, interpretation
from, 3334
mature flow Doppler era, 44, 4647
nondirectional flow Doppler
technique, 2829
peripheral artery recordings, lessons
from, 31, 33
preflow Doppler era
invasive procedures in, 25
noninvasive procedures in, 2526,
26f, 27f
pulsed Doppler with 2D
echocardiography,
combination of, 3637, 38f40f
transcutaneous approaches, return
to, 3436
Early gadolinium enhancement
(EGE), 2020
Index
pediatric, 6
persistent left superior vena cava,
1958
progress in, 5f
right atriumright ventricle, 1958
risk stratification, 1969
superior vena cava, 1958f
supraventricular tachycardia, 1957
supraventricular tachycardia, 1957
Thebesian valve, 1958
therapeutic, 1957
thoracic aorta, 1958f
transesophageal echocardiogram,
1957
transthoracic, 1969
transthoracic echocardiogram, 1957
tricuspid valve, 1958
valve of Vieussens, 1958
Echocardiography, in elderly, 19211956
aortic aneurysm, 19341937
cystic medial degeneration and, 1936
etiology of, 1936
natural history of, 1937
with rupture, 1936f
aortic atherosclerosis, 19211923,
1922f
aortic dissection, 19371942,
1937f1938f, 1941f
acute coronary syndrome and, 1940
color Doppler flow in, 1942
descending thoracic aorta, 1942
perfusing lumen, 1942
rupture, 1938f1940f
aortic stenosis, 19241934
aortic valve area, estimation of, 1926
2D echocardiography, 19241928,
1925f, 1927f
LFLG-AS with low EF, 1929f
live/real time 3D TTE, 19301933
mild, 1921f
PLFLG-AS with preserved EF,
19291930
prevalence and pathophysiology,
1924
severe, 1931f1932f
transcatheter aortic valve
replacement, 1934, 1935f
ventricular response to, 19331934
aortic valve sclerosis, 19231924,
1923f
bicuspid, 1931f
echocardiographic findings for,
19231924, 1930f
normal hemodynamics in, 1924
pathophysiology of, 19231924
prevalence of, 19231924
systolic murmur, 1923
coronary stenosis, 19431946, 1944f,
1946f
left ventricular mass, 19421943
mitral annular calcification, 1946
1948
overview, 1921
penetrating aortic ulcer, 19211923
prosthetic valves, 19481949, 1949f
Echocardiography, in women, 18861920
breast implant, 1887f
congenital heart disease, 19001902
atrial septal defects, 19001901,
1900f1901f
pulmonary hypertension, 19011092
echocardiographic measurements
vs. technical considerations, 1886
1888
ischemic heart disease, 18891893
overview, 1886
polycystic ovarian syndrome, 1899
stress echocardiography, 18941899
structural heart disease, 18881889
mitral valve calcification, 1889, 1895f
mitral valve prolapse, 1888,
1888f1889f
mitral valve stenosis, 18881889
Takotsubo cardiomyopathy, 1899
1900
Echocardiography training, 750760. See
also Training, in echocardiography
Echocardiography transducer, 55
Echo-clear space, 1440f
Echo-guided pericardiocentesis, 1443
1444
EchoPAC, 385
ECMO circuit. See Extracorporeal
membrane oxygenator (ECMO)
circuit
Edge-to-edge repair, 539
Edler, Inge, 45
Edwards Sapien Valve, 2057
Effective orifice area (EOA)
in calcific aortic stenosis, 900
patient prosthetic mismatch and,
10911092
of prosthetic valves, 10841087
Effective regurgitant orifice area (EROA),
518519, 992, 1038
Effusive-constrictive pericarditis, 1448
Eisenmenger syndrome, 1597f
Ejection fraction postamyocardial
infarction, 1165t
I-XV
measurement of flow-mediated
dilatation, 457, 457f
proximal and distal occlusions,
456457
quantification of shear stress, 457
technique of FMD in brachial artery,
458t
factors affecting, 451, 451t
future directions, 471, 474
NO release and, 455, 455f
other methods for endothelial
function assessment, 465
laser Doppler flowmetry, 469470
low flow-mediated vasoconstriction,
470471, 470f474f
peripheral arterial tonometry,
467469, 468f, 469f
pulse wave velocity analysis, 466467,
466f, 467f
shear stress and flow-mediated
dilatation, 452454, 452f
shear stress and FMD response,
analysis of, 457458, 457f
vasoactive molecules in
vasoregulation, 454
endothelins, 454
endothelium-dependent
hyperpolarizing factor, 454
nitric oxide, 454
prostacyclin, 454
Endothelial progenitor cells (EPCs), 450,
450f
Endothelin (ET), 454
Endothelium-dependent hyperpolarizing
factor (EDHF), 454
Endovascular Valve Edge-to-Edge Repair
Study (EVEREST II) trial, 539
End-stage renal disease (ESRD), 1871
1872, 1872f
End-systolic volume index (ESVI), 180,
1379
ICM/NICM and, 14211422
Energy Doppler. See Power Doppler
Enhancement, 61
eNOS (type-III NO-synthase), 454
EOA. See Effective orifice area (EOA)
Epiaortic ultrasonography (EAU),
638641
in aortic pathology
aortic atherosclerosis, 640641, 641f
aortic dissection, 641
epiaortic probe and preparation,
638639, 639f, 640f
imaging views/planes, 639640, 640f
indications for, 638
three-dimensional, 641
I-XVI
F
Fabry disease, 352, 14011402, 1401f
False lumen (FL), 1977f
FAPS. See French Aortic Plaque in Stroke
(FAPS) study
Fast Fourier transform (FFT), 67, 113
Fat pad, 170
Fatty infiltration of liver, 318
FDA 510 (K) Track 3 regulations, 111
Feigenbaum, Harvey, 6
Feinstein, Steve, 13
Fetal bradycardia, 1545
Fetal cardiac function, assessment of, by
velocity vector imaging, 390391
Fetal cardiac imaging, 15271560
congenital heart disease, family
history of, 1529
fetal bradycardia, 1545
fetal cardiac evaluation, indications
for, 1528
fetal cardiology, scope of, 15271528
fetal echocardiography, indications
for, 1529
fetal heart disease, 15291530
congenital cystic adenomatoid
malformation, 1530
congenital diaphragmatic hernia,
1530
sacrococcygeal teratoma, 1530
twin reverse arterial perfusion, 1530
twintwin transfusion syndrome,
15291530
fundamentals of, 15301531
caval long-axis view, 1541
core and cord Dopplers, 1542
ductal and aortic arch views, 1541
fetal cardiac function, 15411542
fetal ultrasound, safety of, 15461547
four-chamber view, 15311541
rhythm assessment, 15421545
short-axis views, 15401541
three-dimensional imaging, 1549
1553
ventricular long-axis view, 1541
overview, 15271560
physiology, 15281529
prenatal counseling in, 1528
reasons and associations for, 1529
1530
Fetal echocardiography, in women,
19061919, 1906f1909f
Fetal heart abnormality, 1529
Fetal heart disease, fetal imaging,
15291530
congenital cystic adenomatoid
malformation, 1530
congenital diaphragmatic hernia,
1530
sacrococcygeal teratoma, 1530
twin reverse arterial perfusion, 1530
twintwin transfusion syndrome,
15291530
FFT analyzer, 67
Fibrinous adhesions, 14561457
Fibroma, 2051
Fibromuscular collar, 1625
Fibrosarcomas, 1488
First pass imaging (perfusion), 2020
Fixed anatomical obstruction, TGA and,
16601661
Fixed subaortic obstruction, 16241625
Flail tricuspid valve, 10071029
3d transthoracic echocardiography,
1012f
in male with dyspnea, 1012f
Flial posterior mitral valve leaflet, 853f
Flow convergence method, MR severity
and, 870871
Index
Flow image, 71
Flow-mediated dilatation (FMD),
452454, 452f454f. See also
Endothelial dysfunction
clinical utility of, 465, 466t
factors affecting
ACE-inhibitors, 465
age and sex, 463
coronary risk factors, 463464
diet, antioxidants, and supplements,
465
exercise, 465, 465f
hyperglycemia, 464
infections, 465
smoking, 464, 464f
sympathetic over activity, 465
unstable angina, 464465, 465f
weight loss, 465
FMD response, 452
Focus, and TEE image quality, 106, 106f
Fontan procedure, and HV Doppler, 318,
318f
Food and Drug Administration (FDA),
13, 110
Fossa ovalis
anatomy of, 533, 535f
2D transesophageal examination,
988f
Fossa ovalis atrial septal defect, 1586
for percutaneous device closure, 1591
Four-chamber asymmetry, 1548f
Fourier, Joseph, 68
Fractional area change (FAC), 1166
Frame, 59
Frame rate, 59
depth and, 59, 60f
image line density and, 59
sector angle and, 59, 60f
Framingham Heart Study, 1161
French Aortic Plaque in Stroke (FAPS)
study, 1921
Frequency, 55
in clinical use, 57
and TEE image quality, 104105, 105f
Fully sampled matrix-array transducers,
77, 268
Functional mitral regurgitation (FMR),
1372
DCM and, 1376, 1376f
Fundamental imaging, 57
apical four-chamber view, 57f
G
Gall stones, 220
Gating, 80
H
Haemophylus influenzae, 1043
Hand-carried ultrasound device (HCD),
752
Harmonic imaging, 57
apical four-chamber view, 57f
HeartAssist system, 1228
Heart chamber segmentation algorithms,
706707, 707f
Heart defects, in fetus, 15271528
Heart failure with preserved ejection
fraction (HFnlEF), 1122
HeartMate II, 694, 1228
HeartWare ventricular system, 1224f,
1228
Heating safety, TEE probe equipment
and, 111
Hemangioma
involving mitral valve, 1485f
left atrial, 1487f
right ventricular, 1486f
Hematoma, 1517f
after femoral artery access, 696, 697f
Hemochromatosis, 1405
Hemodynamometer, 67
Hepatic veins (Hep V), 1532f
anatomy of, 299300, 300f
versus biliary ducts, 305, 305f
blood flow in, 300, 301f
aging and, 303
Doppler V wave, 300, 302f
exercise and, 303
factors affecting, 302303, 303f
gender and, 303
HV Doppler measurements, 302, 302f
normal, 300, 301f
physiology of, 300302, 301f, 302f
pregnancy and, 303
respiration and, 302303, 303f
two components of systolic flow, 300,
301f
challenge to use of HV Doppler,
319321, 320f
disease states, and HV flow pattern,
305
atrial fibrillation, 309, 309f
atrioventricular dissociation, 308,
308f
cardiac tamponade, 317, 317f
cardiopulmonary bypass, 317, 318f
chronic obstructive lung disease, 306,
307f
congenital heart disease, 317318,
318f
constrictive pericarditis, 315317,
316f, 317f
liver disease, 318319, 319f
premature ventricular contractions,
309, 309f
prolonged PR interval, 308, 308f
pulmonary hypertension, 311, 313,
313f
restrictive cardiomyopathy, 315, 315f
right ventricular diastolic
dysfunction, 313315, 315f
right ventricular end-diastolic
pressure, assessment of,
309310, 310f
right ventricular systolic dysfunction,
313, 314f
short PR interval, 307, 308f
sinus bradycardia, 306, 307f
sinus tachycardia, 306, 307f
tricuspid regurgitation, 310311, 311f,
312f
I-XVII
tricuspid stenosis, 311, 312f
heterotaxy syndrome and, 1706
HV Doppler flow patterns, schematic
drawing of, 321f
imaging of, 299302
pulse Doppler across, 1539f
spectral Doppler of, 319321
versus superior vena cava, Doppler
pattern of, 304, 304f
transesophageal echocardiography,
305
transthoracic echocardiography, 304,
305f
visualization and recording of HV
Doppler, technical considerations in,
305, 305f, 306t
Hepatic vein systolic filling fraction
(HVFF), 1268
Hertz, Hellmuth, 45
Heterotaxy syndrome, 17041709
anatomy, 1704
cardiac and extracardiac anomalies
in, 1705t
initial echocardiogram of, 17041709
abdominal situs and cardiac position,
17041705
aortic arch and branches, 1709
atria and appendages, 1707
atrial septum, 1707
atrioventricular junction, 17071708
branch pulmonary arteries, 1708
1709
pulmonary blood flow, sources of,
17081709
venoatrial connections, 17051707
ventricles and ventricular septum,
1708
ventriculoarterial connections, 1708
overview, 1704
venoatrial connections in, 1706t
Hewlett Packard SONOS 5500, 227
HFnlEF. See Heart failure with preserved
ejection fraction (HFnlEF)
High-flow, high-gradient aortic stenosis,
920. See also Aortic stenosis
Highly focused ultrasound (HIFU), 1996
High MI imaging, 420421
High-pass filtering, 6667
High pulse repetition frequency (HPRF),
64
HIV-associated cardiomyopathy, 1407
HLHS. See Hypoplastic left heart
syndrome (HLHS)
HOCM. See Hypertrophic obstructive
cardiomyopathy (HOCM)
Hodgkin lymphoma, 1491
I-XVIII
I
ICE. See Intracardiac echocardiography
(ICE)
ICM. See Ischemic cardiomyopathy
(ICM)
IEC 60601-1 document, on safety of
medical electronic devices, 110
Image line density, and frame rate, 59
Image optimization and equipment, 60
depth and, 61
focus and, 61
gain and, 61
sector width and, 61
zoom and, 61
Image rendering, in 3D echocardiography
2D tomographic slices, 7980
surface rendering, 79
volume rendering, 7879, 79f
Imaging artifact, 3D reconstruction
imaging and, 17
IMH. See Intramural hematoma (IMH)
Impella catheter-based assist device, 1227
Impella device, 1250, 1251f
Individual mitral lesions, evaluation of,
16111615
abnormal mitral arcade, 16131614
accessory mitral orifice, 1615
cleft mitral leaflet, 1613
double orifice mitral valve, 1614
1615, 1614f
dysplasia of mitral valve, 1613
Ebsteins anomaly of mitral valve, 1615
hypoplastic mitral valve, 1613
mitral valve prolapse, 1615
parachute mitral valve, 1613
supramitral ring or membrane,
16111613
Indocyanine green, 11
Index
abnormalities of, 16821683
bilateral, 1683
inferior vena cava interruption,
16821683
inferior vena cava to left atrium, 1683
cannula position in, 1252f
dilated, 1066f
in dilated cardiomyopathy, 1373
1374, 1374f
hepatic vein, 1964
heterotaxy syndrome and, 17051706
inspiratory collapse, 1964
plethora (dilation) of, 1441f
pulse Doppler across, 1539f
for RAP, 12641266, 1266f
size, 1964
subcostal window, 1964
thrombi, 1966
transgastric level, 1964
ventricular assist devices and, 1233
Inferior vena cava interruption, 1682
1683
Ingenious balloon, 535
Inkjet technology, 6
Inlet VSD, 1659
Instrumentation, Doppler, 6668
Interagency Registry for Mechanically
Assisted Circulatory Support
(INTERMACS), 1224, 1226
Interatrial communication, TGA and,
1657
Interatrial membrane, color Doppler
across, 1532f
INTERMACS. See Interagency Registry
for Mechanically Assisted
Circulatory Support
(INTERMACS)
Internal carotid artery (ICA), 665668
International Electrotechnical
Commission (IEC), 110
International Registry of Acute Aortic
Dissection study, 980
International Society of Cardiovascular
Ultrasound (ISCU), 753, 758
Interrupted aortic arch, 16941695, 1695f
classification of, 1695t
type B arch interruption, 1694
Intersocietal Commission for the
Accreditation of Echocardiography
Laboratories (ICAEL), 132
Interventricular septum (IVS), 1534f
Intra-aortic balloon pump, and carotid
ultrasound findings, 693
Intracardiac echocardiography (ICE),
643653
advantages of, 652
clinical applications, 644
during electrophysiology intervention
atrial septal puncture, 644645,
646f
EP procedures and, 647648
pulmonary vein isolation for atrial
fibrillation ablation, 645, 647,
647f
right ventricular outflow tract
tachycardia, 647, 648f
ventricular tachycardia from left
ventricle, 647, 649f
VT foci from great vessels, 647,
648f649f
equipment and catheters, 643
mechanical system, 643
phased array ultrasound system, 644,
644t
extracardiac use of, 651, 652f
imaging specifications, 644, 645f
limitations of, 652
during structural intervention
Melody valves, 651
patent foramen ovale/atrial septal
defect closure, 648, 650, 650f
periprosthetic valve, 650651, 651f
ventricular septal defect closure, 650,
651f
three-dimensional, 652, 653f
Intracardiac hardware, 15141517
Intramural hematoma (IMH), 957958,
958f
transesophageal imaging of, 978
Intrauterine growth retardation (IUGR),
1528
Intravascular ultrasound (IVUS), 643,
655661
examination procedure, 656657
future perspectives, 661
image acquisition, 655656
electronically switched multielement
array system, 656
mechanically rotating transducer,
655656
image interpretation, 657659, 658f,
659f
safety of, 661
technology, 655
utility of, 659660, 660f
Ischemic and nonischemic
cardiomyopathy
cardiac magnetic resonance imaging,
14271428
cronary computed tomographic
angiogram, 1427
I-XIX
doppler echocardiography, 1421
1424
coronary echocardiography, 1424
left atrial size, 1422
left ventricular diastolic dysfunction,
1422
left ventricular end-systolic volume
index, 14211422
left ventricular volumes, 1421
mitral regurgitation, mechanism,
14221424, 1423f
myocardial contrast
echocardiography, 1424
right ventricle dysfunction, 1422
stress echocardiography, 1424
tenting area, 1422, 1423f
echocardiographic assessment, 1419
echocardiographic differentiation of,
14181434
large mitralseptal separation, 1419
M-mode echocardiography, 1419
1421
overview, 1418
positron emission tomography, 1428
severely dilated left ventricular cavity,
1419
single-photon emission computed
tomography, 14251427
Ischemic cardiomyopathy (ICM), 1388
1395, 1395f, 1418, 1420ff1421f
vs. NICM, 1425, 1426t
Ischemic cascade, 1307f
Ischemic handgrip, 453
Ischemic heart disease, 12891305
central mitral regurgitation and,
1298f
chronic. See Chronic ischemic heart
disease detection of, 12891292
pharmacological stress testing, 1290
stress echocardiography for, 1289
1291, 1290f
vasodilator stress testing, 1290
echocardiography in
myocardial contrast stress, 12911292
speckle-tracking, 13011303, 1303f
three-dimensional, 13011302
overview, 1289
Ischemic LV dysfunction, 3D speckle
tracking and, 376t
Ischemic mitral regurgitation, 858863
Isolated carditis, 774775
Isolated mitral valve cleft, 1775
Isomerism, echocardiography of, 1577
Isomerism syndromes. See also
Heterotaxy syndrome
I-XX
cardiac and extracardiac anomalies
in, 1705f
venoatrial connections in, 1706t
Isovolumic contraction (IVC), 11851186
Isovolumic contraction time (IVCT), 1140
Isovolumic relaxation time (IVRT), 313,
1069t, 1140
IUGR. See Intrauterine growth
retardation (IUGR)
IVC. See Inferior vena cava (IVC)
IVCCI. See IVC collapsibility index
(IVCCI)
IVC collapsibility index (IVCCI), 1265
IVCT. See Isovolumic contraction time
(IVCT)
IVRT. See Isovolumic relaxation time
(IVRT)
IVUS. See Intravascular ultrasound
(IVUS)
IVUS with virtual histology (IVUS-VH),
657
J
Jarvik-2000 Flowmaker, 1228, 1228f
Joyner, Claude, 6
Junctional rhythm, and PV Doppler, 333,
334f
K
Kaul, Sanjiv, 13
Kawasaki disease (KD), 16881689
in childhood, 1861
coronary arteries with, 1862t
velocity vector imaging in, 398
L
LAD. See Left anterior descending (LAD)
LaGrangian strain, 389
Lambls excrescences, 1015, 1480f
LAP. See Left atrial pressure (LAP)
Large intracardiac thrombus, 1978
atrial appendage, 1978
atrial fibrillation, 1978
atrial flutter, 1978
autopsy, 1979
deep venous thrombosis, 1979
disystole, 1978
echolucent, 1979
endocardium, 1978
heart embolism, 1978
intracardiac devices, 1979
systole, 1978
transthoracic echo, 1979
transthoracic images, 1979
ventricular apex, 1979
Lariat procedure, 563, 566, 566f568f
Laser Doppler flowmetry (LDF), 469470
Laser Doppler perfusion monitoring
(LDPM). See Laser Doppler
flowmetry (LDF)
Late gadolinium enhancement (LGE),
2003, 2020
Latent diastolic dysfunction, stress
echocardiography in, 1318
Lateral resolution, 59, 59f, 733, 734f
Lavengin, Paul, 4
LBBB. See Left bundle branch block
(LBBB)
Lead infection, associated with
pacemaker, 11141115
Lead perforation, 12151217
chest CT for, 1216
echocardiography in, 1216f
reported cases of, 1217t
Lead zirconate titrate (PZT), 1992
Leakage current., 110111, 111f, 111t
Left anterior descending (LAD), 1337
artery disease, 1328
distal post-stenotic diastolic-to systolic velocity ratio, 13401341
Left atrial appendage (LAA), 1960
abnormalities, 1960
adequate visualization, 1960
clot detection 3D TEE for, 512, 619
continuous wave, 1963f
3DE assessment of, 277, 277f
doppler interrogation, 1960
embolism, 1960
endocardial device closure of,
561563, 562f565f
epicardial suturing of. See Lariat
procedure
exclusion, 1961
percutaneous, 561
surgical, 560561
mid-diastolic signals, 1960
mitral valve, surgery, 1960
orthogonal mass abnormality, 1960
pectinate lines, 1960
persistence, 1961
prominent ridges, 1960
recanalized, 1963f
thrombus, 1960
traverse, 1962f
Left atrial appendage clot, embolization
of, 1917f
Left atrial appendage thrombus, 2058f
Index
left upper pulmonary vein, 1962f
M-mode echocardiography, 1960
pectinate muscles, 1962f
pulmonary veins, 1960, 1961f
rhythm analysis clues, 1960
spontaneous echo contrast, 1960
swirling appearance, 1960
thromboembolic, 1960
complications, 1960
event, 1960
nonvalvular, 1960
risk, 1960
valvular, 1960
transesophageal echocardiogram,
1961f
volume, 1960
Simpsons biplane method, 1960
Left atrium myxoma, 2053f
Left bundle branch block (LBBB), 1217,
1377
Left heart hemodynamics, age groups for,
1275t
Left isomerism, 1577
Left lower pulmonary vein (LLPV), 327,
328f
Left main coronary artery (LMCA)
flow in distal, detection of, 1340
transesophageal echocardiography,
13381340, 1339f
transthoracic echocardiography,
13371338, 1339f
Left main coronary artery ostium, 647
Left main coronary artery stenosis
(LMCAS), 1325
Left parasternal approach, of three dimensional echocardiography,
244, 245f254f
Left-sided filling pressures, 12731279
color M-mode flow propagation
velocity, 1278
echocardiographic methods for,
1273t1274t
evaluation of, 1277f
variables used for, 1278t
left atrial dimensions, 12781279
mitral inflow parameters in, 1274
1276, 1275f, 1275t
pulmonary venous flow and, 1276,
1276f
tissue Doppler annular diastolic
velocities, 12771278
Left-sided superior vena cava (LSVC),
1535, 1545f
Left upper pulmonary vein (LUPV), 327,
328f, 1962f
I-XXI
computed tomography and, 2063
2064
left ventricular overfilling, 12401246
causes, 1245t
mitral flow prior to placement of,
1243f
net forward cardiac output and,
12471248
optimizing settings, 12481249, 1250f
placement response of, factors affect,
1235f
power failure of, 1244f
structures in patient with, 1239f
transesophageal echo, postoperative,
1237f
Left ventricular diastolic dysfunction
grades of, 1127f
ICM/NICM and, 1422
left atrial dilation in patient with,
1125f
Left ventricular diastolic function
2D echocardiography, 11241125
flow propagation velocity, 1127
color Doppler, 1129f
future directions for, 11311132
imaging techniques for, 11311132
integrating echocardiographic
variables, 11301131, 1130f1131f
left atrial size, 11241125
left ventricular mass, 11241125
in nonsinus rhythm, 11291130
overview, 1124
pulmonary artery pressure, 1129
pulmonary artery systolic pressure
and, 1130f
pulmonary venous flow, 11271128
tissue Doppler imaging for, 1127,
1128f
Valsalva maneuver, 11261127
wall function, 11241125
Left ventricular dimensions, 19421943
Left ventricular dysfunction, ultrasound
stethoscope for screening of, 295
Left ventricular dyssynchrony, 1218
three-dimensional speckle tracking
and, 376t
Left ventricular ejection fraction (LVEF),
381, 1116, 1117, 11241125
in LFLG-AS, 1929
pacemaker and, 1218f
systolic myocardial dysfunction and,
1131f
Left ventricular end-diastolic
diameter (LVEDD), 1886
I-XXII
Index
myocardial performance index, 1120
overview, 1115
Quinones method, 1117
seventeen-segment model, 1116f
sphericity index, calculation of, 1119
Tei's index, 1120, 1120f
tissue Doppler imaging, 1119
transthoracic echocardiography
three-dimensional, 1122
two-dimensional, 11161119
velocity vector imaging, 1120
visual estimation of, 11151116
Left ventricular thrombus, 15041506
Left ventricular volumes
assessment of, 81
in ICM/NICM, 1421
Leiomyosarcoma, 627
aortic, 1489f1490f
left atrial, 1488f
Levacor ventricular assist device, 1228
Levo-transposition of great arterie, 1759f
Levo transposition of great vessels, in
female, 1916f
LFLG-AS. See Low-flow, low-gradient
aortic stenosis (LFLG-AS)
LibmanSacks endocarditis, 18671868,
1868f
Libman-Sacks vegetations, 931
Lidocaine, 508
Light and sound, 1995, 1996
acousto-optical imaging, 1996
complementary approach, 1996
endogenous absorbers, 1995
laser pulses, 1995, 1996
precision, 1995
photoacoustic imaging, 1995
Schlieren tank, 1996
classic implementation, 1996
Linear array system, 8
Linear/phase array transducers, 76, 76f
Linear probe, 58, 58f
Line density, 60
Lipoma, 2054f
Lipomatous hypertrophy
of atrial septum, 1484f
of interatrial septum, 182, 182f, 533,
536f, 2054, 2056f
Live 3DE, 269
Liver cirrhosis, and HV Doppler, 318, 319f
Liver disease, and HV Doppler, 318319,
319f
LMCA. See Left main coronary artery
(LMCA)
LMCAS. See Left main coronary artery
stenosis (LMCAS)
cardiogenic watery B-lines, 1982,
1983
differential diagnosis
challenge, 1982
extravascular lung water, 1982
human acute respiratory distress
syndrome, 1983
limitations, 1987
B-line interpretation, 1987
obese patients, 1987
patient-dependent, 1987
lung sliding, 1982
main approaches, 1983
methodology, 1983, 1984
pulmonary parenchyma, 1983
thoracic scanning areas, B-lines
semiquantitative assessment,
1983, 1984f
two-dimensional scanner, 1983
physical basis, 1982, 1983
physical scatterer, 1982
physiological basis, 1982, 1983
pulmonary edema, 1983
sonographic appearance, 1982, 1983f
consolidated lung, 1983f
multiple B-lines, 1982, 1983f
normal lung, 1983f
LUPV. See Left upper pulmonary vein
(LUPV)
LVAD. See Left ventricular assist device
(LVAD)
LV concentric hypertrophy, 920
LV contractility, reduction in, 920
LV diastolic function, assessment of, by
TD imaging, 352353, 355f, 356f
LV dyssynchrony, TD Imaging for, 353,
354f
LVEDD. See Left ventricular end-diastolic
diameter (LVEDD)
LVEDP. See Left ventricular end diastolic
pressure (LVEDP)
LVEDV index, 1157
LVEF. See Left ventricular ejection
fraction (LVEF)
LV ejection fraction (LVEF), 270
in acute coronary syndromes,
12931294
LVESD. See Left ventricular end-systolic
diameter (LVESD)
LVESV index, 1157
LV filling pressures, estimation of, by TD
imaging, 352353
LVIDd. See Left ventricular internal
dimension in diastole (LVIDd)
I-XXIII
M
MAIN-COMPARE trial, 659
Major adverse cardiovascular events
(MACE), 2042
Malignant melanoma, 1500
Malignant primary cardiac tumors,
14841511
angiosarcoma, 14851486
cardiac plasmacytoma, 14901491,
1492f
fibrosarcomas, 1488
hydatid cyst, 1491, 1493f1498f
pericardial mesotheliomas, 1491
primary cardiac lymphoma, 1490
rhabdomyosarcoma, 14861488
sarcomas, 14841488
Manometers, 27
Marfan syndrome (MFS), 958959
TAA and, 1936
Matrix biplane transesophageal echo
(TEE) probe, 99100, 100f
MDCT. See Multidetector computed
tomography (MDCT)
I-XXIV
Index
pap muscle dysfunction, 1972
primary, 880883
leaflets, 880883
mitral annulus, 883
subvalvular apparatus, 883
pulmonary venous flow, 886
and PV Doppler, 336338, 337f, 338f
rupture, 1972
secondary, 883884
left ventricular remodeling, 883884
mitral annulus, 883
mitral valve distortion, 883884
severity of, 885889
grading of, 888
qualitative assessment of, 885886
quantitative assessment of, 886888,
888f
semiquantitative assessment of, 886
surgical correction of, 538540, 541f,
542f
transesophageal echocardiography,
1972
vena contracta, 886
Mitral regurgitation, echocardiographic
assessment of, 847863
causes of, 848t
echocardiography, role of, 848t
etiology and mechanism of, 848849
hemodynamic consequences,
874876
left atrial size and pulmonary
pressures, 876
left ventricle size and function,
874876
key notes, 847849
mitral valve prolapse, 849
severity of, 863876
antegrade velocity of mitral inflow,
869
color Doppler jet area, 863868
continuous-wave jet intensity, 869
echocardiographic assessment of,
864t
PISA method, 870871
pulmonary venous flow, 869
quantitative echo/Doppler methods,
869874
semiquantitative echo/Doppler
methods, 863869
vena contracta width, 868869
surgical considerations in, 876
Mitral stenosis, 776791
anterior mitral valve leaflet, 777, 780f
associated lesions, 783784
cardiac MRI for, 2013
continuity equation, 789
3DE assessment of, 280, 281f, 282
echocardiography
exercise, 790
pitfalls of using, 790791
recommendations for, 778t
three-dimensional, 791
two-dimensional, 778779, 779f
mitral valve, assessment of, 779783
abnormal motion of leaflets, 779781
commissural fusion, 781783
PISA method, 789
planimetry, 785
pressure gradient, 785787
pressure half-time, 787789
and PV Doppler, 338339, 339f
severity
classification of, 784t
determination of, 784785
indices of, 789790
methods for quantification of, 785t
Mitral stenosis, echocardiographic
assessment, 826847, 827f
anatomic considerations, 826827
atrial septal defects, 842
balloon mitral valvotomy, 839t, 842f
complications of, 840841
echocardiography in patients for,
838840
evaluation of patient, 838840, 839t
mitral regurgitation in, 840841
post, 841f, 843f, 844f
cardiac perforation, 841842
causes of, 846847
conditions clinically mimicking
rheumatic, 844846
atrial ball valve thrombus, 845
congenital double orifice mitral
valve, 844845
congenital mitral stenosis, 844
congenital parachute mitral valve,
845, 846f
degenerative mitral valve stenosis,
844
left atrial myxoma, 845
diseases of other valves, 834836
echocardiography
in balloon mitral valvotomy, 838840,
839t
exercise, 844
role of three-dimensional, 844
two-dimensional, parameters of, 827
indices of severity, 829834
continuity equation, 833
diastolic pressure gradient, 829831
mitral valve area planimetry, 831
PISA method, 834
pressure half-time, 831833, 832t,
833f
Wilkins Score, 830t
left atrium, 834
left atrium appendage, 834
long-term outcome, 842843
mitral regurgitation and, 834836
mitral valve morphology, 827829
leaflet motion, 828
subvalvular pathology, 828
valve calcification, 828829
valve thickening, 827828
normal mitral valve area, 827
pulmonary hypertension, 836838
mitral valve echocardiographic
scoring system, 836
Wilkins scoring system, 836837
rheumatic, 827
RT 3DE scoring system, 838t
Mitral valve (MV), 577579, 578f, 579f
in adults, 18181820
cleft, 18181819
cor triatriatum, 1818
double orifice mitral valve, 18191820
echocardiography, 1818
mitral regurgitation, 1820
parachute, 1820
anatomy, assessment of
Cormier score, 782t
Wilkins score, 782t
anatomy of, Cormier Score, 837t
3DE assessment of, 278, 278f, 280,
281f
3D echo of, 516520, 517f, 518f
pulsed wave Doppler across, 1533f
real time 3D echocardiographic score
of, 783t
short-axis, 1533f
Mitral valve, congenital anomalies of,
16101615
individual mitral lesions, evaluation
of, 16111615
abnormal mitral arcade, 16131614
accessory mitral orifice, 1615
cleft mitral leaflet, 1613
double orifice mitral valve, 1614
1615, 1614f
dysplasia of mitral valve, 1613
Ebsteins anomaly of mitral valve,
1615
hypoplastic mitral valve, 1613
mitral valve prolapse, 1615
parachute mitral valve, 1613
I-XXV
supramitral ring or membrane,
16111613
mitral valve lesions,
echocardiographic views,
16101611
types of, 1611t
Mitral valve area (MVA), 282
Mitral valve calcification, 1889
in women, 1889
Mitral valve diseases, 775776, 826879,
1318
anatomy of mitral valve, 775776
mitral regurgitation, 791801
assessment of, 793
color Doppler flow, 793796
continuous wave Doppler, 796797
exercise echocardiography, 800
follow-up in, 801
pulmonary vein flow, 798
pulsed Doppler, 797798
severity of, 793
integrative approach for, 800801
organic, 801t
role of TEE in assessing, 788800,
799t
supportive signs, 798
three-dimensional
echocardiography, 800
two-dimensional echocardiography,
792793
mitral stenosis, 776791
overview of, 826
role of 3D TEE in operating room in,
577582, 578f593f
stress echocardiography in, 1318
Mitral valve insufficiency, 1860f
Mitral valve lesions, echocardiographic
views, 16101611
Mitral valve morphology, 827829
leaflet motion, 828
subvalvular pathology, 828
valve calcification, 828829
valve thickening, 827828
Mitral valve opening, and untwisting,
12001201
Mitral valve orifice area (MVOA), 777,
790791
continuity equation, 789
mitral PHT and, 787789
Mitral valve performance, 1249
Mitral valve prolapse, 169
3D TTE and, 282, 282f
ultrasound stethoscope for screening
of, 295
Mitral valve prolapse (MVP), 849863,
1615
I-XXVI
echocardiographic assessment of,
850855
M-mode, 850
three-dimensional, 851855
two-dimensional transesophageal,
851, 855f, 856f
two-dimensional transthoracic, 851,
852f855f
surgical methods and indicators in,
855863
degenerative mitral regurgitation, 863
functional mitral regurgitation,
857858
infective endocarditis, 863
ischemic mitral regurgitation,
858863
rheumatic mitral regurgitation, 863
tricuspid and pulmonary valve
prolapse with, 855f
in women, 1888, 1888f1889f
Mitral valve quantification
three-dimensional echocardiography
and, 8182, 82f, 83f
Mitral valve segmentation analysis, 881f
Mitral valve stenosis, in female, 1888
1889
Mitral valve vegetation, in female, 1911f
Mitral valvular verrucous nodules, 773f
M-mode echocardiography, 3, 3f, 58, 58f,
119130
development of, 47
in left ventricular systolic function,
11161119
E point septal separation, 1117
left ventricular ejection fraction,
1116, 1117
parasternal long-axis, 1116
in right ventricle, 11361138
transthoracic echocardiogram, 136,
136f
of tricuspid valve, 984986
Ebsteins anomaly, 986f
functional events, demonstrating,
984f
septal leaflet, identification, 985f
systolic abnormalities, 986f
using contrast injections,
identification, 985f
Moderator band, 1503
MPAP. See Mean pulmonary artery
pressure (MPAP)
mPAP. See mean pulmonary artery
pressure (mPAP)
MSCT. See Multislice computed
tomography (MSCT)
99mTc-sestamibi gated single photon computed emission
tomography (GSPECT), 1157,
1159f
Mucopolysaccharidosis, 931
MullinsTM catheter, 533
Multi-beat acquisition, in 3D
echocardiography, 7475, 75f
Multicenter Aneurysm Screen Study
(MASS), 294
Multidetector computed tomography
(MDCT), 2023
acute chest pain evaluation with,
20452047
for coronary artery anomalies,
20472049
techniques of, 2047
Multigate pulsed Doppler, 70
Multipath reflection, 81
Multiplane mode, 268269
Multiple-beat 3DE imaging, 269
Multiple thrombi, with poor ventricular
function, 1377f
Multislice computed tomography
(MSCT), 1427
Muscular inlet defect, 1593
Muscular outlet defect, 1593
Muscular trabecular defect, 1594
Muscular ventricular septal defects,
557558, 15931594, 1593f, 1594f.
See also Ventricular septal defects
(VSDs), closure of
muscular inlet defect, 1593
muscular outlet defect, 1593
muscular trabecular defect, 1594
MV clipping, 538540, 541f, 542f
3D TEE guidance of, 540
MitraClip device for, 539
patient selection for, 540
MVOA. See Mitral valve orifice area
(MVOA)
MVP syndrome (MVPS), 1888
MWFS. See Midwall fractional shortening
(MWFS)
Myocardial blood flow (MBF), 442
Myocardial blood volume (MBV), 422
Myocardial contractile motion, 1159
1160
Myocardial contractile velocity, 1119
Myocardial contrast echocardiography
(MCE), 416, 422, 430. See also
Contrast echocardiography
for CAD detection, 430431
coronary flow reserve by, 431
for myocardial perfusion assessment,
427428, 429f
Index
for myocardial viability detection, 431
Myocardial contrast stress
echocardiography, 12911292
Myocardial deformation imaging, 360.
See also Strain imaging
Myocardial disease, detection of, by TD
imaging, 350352
Myocardial fiber organization, 1180f
Myocardial infarction
mechanical complications of,
12941298
left ventricular aneurysm, 12981300
left ventricular free wall, rupture of,
1294
left ventricular pseudoaneurysm,
12941295
papillary muscle rupture, 1297, 1297f
ventricular septal rupture, 1295
1297, 1296f
speckle-tracking echocardiography,
1302
vs. cardiac death, 13111315
Myocardial ischemia, speckle-tracking
echocardiography, 1302
Myocardial perforation, pacemaker
associated, 12151217
2D transesophageal
echocardiography in, 1216,
1216f
reported cases of, 1217t
RT3DE in, 1216f
Myocardial performance index (MPI),
1120
ICM/NICM and, 1419
in rheumatoid arthritis, 1868
Myocardial perfusion
echocardiography, 441447
acute coronary syndromes, 443
chronic coronary artery disease,
443445
myocardial perfusion evaluation, by
CE, 441443
nonischemic dilated
cardiomyopathy, assessment
of, 445446, 447f
and stress echocardiography, 1319
Myocardial tagging, 405
Myocardial viability, speckle-tracking
echocardiography, 13021303
Myocardial viability assessment, contrast
echocardiography for, 443
Myocardial wall motion abnormality,
1311
Myocarditis, velocity vector imaging in,
398399
N
Narrow-angled display, 241
National Board of Echocardiography
(NBE), 754
National Electrical Manufacturers
Association (NEMA), 110
National Institutes of Health, 8
Native valve endocarditis, role of 3D TEE
in operating room in, 597604,
609f612f
Nitric oxide (NO), 454
and endothelial function, 450, 451
Nodal re-entrant tachycardia (NRT), 1957
Noninvasive hemodynamic monitoring,
229230, 230f, 231f
Nonischemic dilated cardiomyopathy
(NICM), 1418
mitralseptal separation in, 1419f
vs. ICM, 1425, 1426t
Nonsinus rhythm, 11291130
Nonstandard echocardiographic
examination, 188223
abdominal examination, 220
examination from back, 216, 218, 220
two-dimensional transthoracic
echocardiography, 221f
left atrial appendage examination,
212, 214, 216, 217f219f
live/real-time three-dimensional
transthoracic echocardiography,
217f219f
two-dimensional transesophageal
echocardiogram, 219f
two-dimensional transthoracic
echocardiography, 217f
left parasternal and apical planes for
coronary arteries, 190, 204, 207,
213f216f
live/real-time three-dimensional
transthoracic echocardio
graphy, 214f216f
two-dimensional transthoracic
echocardiography, 213f214f
right and left supraclavicular
examination, 189190,
205f212f
live/real-time three-dimensional
transthoracic echocardio graphy, 209f212f
two-dimensional transthoracic
echocardiography, 205f209f
right parasternal examination planes,
188189, 189f204f
live/real-time, three-dimensional
transthoracic echocardio graphy, 196f204f
two-dimensional transthoracic
echocardiography, 189f196f
Non-ST elevation myocardial infarction
(NSTEMI), evaluation of, 226
No-reflow phenomenon, 443
Normal heart, 11851194
left ventricle, 11851194
isovolumic contraction, 11851186
lengthening, 1189
postejection isovolumic phase,
11881193, 1192f
rapid filling, 11931194, 1193f
recoiling, 1189
torsion, 11861188
sonomicrometer crystal tracings,
1203f
ventricular narrowing, 1187f
Nutritional deficiency, 1880
Nyquist, Harry, 70
Nyquist limit, 69, 70, 736
O
Obesity, and HV Doppler, 318319, 319f
Off-axis imaging, 164
One-dimensional (1D)
echocardiography, 3f, 25, 25f
Optigo, 291, 292f
Optison, 417, 418t. See also Contrast
echocardiography
Ostium primum atrial septal defect, 1586
Outflow tract obstruction, 17661770
congenital aortic stenosis/bicuspid
aortic valve, 17661770
subaortic stenosis, 1770
P
Paced rhythm, and PV Doppler, 334, 334f
PAcT. See Pulmonary flow acceleration
time (PAcT)
PA diastolic pressure (PADP), 230, 231f
I-XXVII
I-XXVIII
two-dimensional anatomic imaging,
142
right ventricle inflow view, 142, 142f,
142t, 143f
Doppler imaging, 142, 143f
technique, 142, 142f
two-dimensional anatomic imaging,
142, 143f
right ventricular outflow tract view,
142144, 143f, 143t, 144f
Doppler imaging, 143144, 144f
technique, 142, 143f, 143t
two-dimensional anatomic imaging,
143, 144f
Paravalvular aortic prosthetic
regurgitation, 1105f
Paravalvular mitral prosthetic
regurgitation, 1102f1104f
Paravalvular mitral regurgitation, in
female, 1918f1919f
Paravalvular prosthetic leaks
closure of, 546548, 549f, 550f
3D TEE monitoring of, 548, 550f, 551f
3D TEE and, 512, 513f
Paravalvular regurgitation (PVR), 608612
of prosthetic valves, 1099
vs. transvalvular regurgitation, 1106
Partial anomalous pulmonary venous
connection (PAPVC), 1672
PASP. See Pulmonary artery systolic
pressure (PASP)
Patches, 78
Patent ductus arteriosus (PDA), 1599
1602, 17511754, 1755f1757f,
18051809, 1808t
anatomy, 1599
aortic runoff, 1601
cardiac catheterization, 1808
chamber dimensions, 1601
closure of, 548550, 551f552f, 1809
direction of shunt, 1601
duct morphology, 16001601
subclavian origin, 1601
usual ductus, 1600
vertical duct, 16001601
ductus, characteristics of, 1600
echocardiography, 15991600
ductal view, 1599
echocardiographic views, 15991600
objectives, 1599
suprasternal view, 15991600
echocardiography for, 18051807
hemodynamic significance of, 1601
with left-to-right shunt, 1807
MRI/CTA, 1809
postoperative adult, 1809
pulmonary arterial pressure, 1601
stepwise evaluation for, 1599t
TGA and, 1659
transesophageal echocardiography,
1808
Patent foramen ovale, 15851586
Patent foramen ovale (PFO), 7
closure of, 555, 557, 558f
ICE imaging during, 648, 650, 650f
color contrast in, 1552f
Patient prosthetic mismatch (PPM),
10911092
Pattern correlation (PC), 88
PAU. See Penetrating aortic ulceration
(PAU)
PA wedge pressure (PAW), 230, 231f
PCWP. See Pulmonary capillary wedge
pressure (PCWP)
Peak filling rate (PFR), 11571158
RT 3DE in, 1158f
Pediatric echocardiography, birth and
development of, 6
Pediatric hearts, imaging of, 285286
Penetrating aortic ulcer (PAU), 961, 962f
in elderly, 19211923
Penetrating chest trauma, 1972
close evaluation, 1972
hemothorax, 1972
impaling object, 1972
transesophageal echocardiogram
(TEE), 1972
wounds, 1972
lower left parasternal, 1972
lower right parasternal, 1972
Percutaneous continuous flow devices,
12501252
Impella device, 1250, 1251f
TandemHeart, 12501251
Percutaneous mitral balloon
valvuloplasty (PMBV), 533537,
537f539f
Percutaneous transvenous mitral
commissurotomy (PTMC), 777,
790
Perfluorocarbon gases, 13
Perfusion Score Index (PSI), 423
Pericardial cysts, 1460f, 2054, 2055f
Pericardial diseases, 14351451
acute pericarditis, 1436
anatomy of, 14351436
computed tomography for, 2064
2066
Index
congenital anomalies, 14481449
constrictive pericarditis, 1444
Doppler flow velocity records,
14441448
M-mode and 2D echo, 1444
3D echocardiography, 14521461
echocardiographic appearance,
14351436
effusive-constrictive pericarditis,
1448
fibrin deposits and, 1456
M-mode and 2D echocardiography,
14371438
multimodality imaging of
pericardium, 1450
overview, 1435
pathophysiology of, 1436
pericardial effusion, 14361437
pericardial tamponade, 14381440
Doppler flow velocity recordings,
14421443
echo-guided pericardiocentesis,
14431444
two-dimensional echo, 14401442
physiology of, 1436
Pericardial duplication cyst, computed
tomography for, 2064
Pericardial effusion, 10451047, 1436
1437
circumferential, 1500f
computed tomography for, 2065,
2065f
3D transthoracic echocardiography
advantages of, 1453t1454t
live/real-time, 1454f, 1455f1456f
vs. 2D transthoracic
echocardiography, 14531456
echocardiography of
M-mode, 1439f
two-dimensional, 1438f
epicardial fat and, 1438
exudative, 1439f
tuberculous, 1458f
Pericardial hydatid cyst, 1497f1498f
Pericardial masses, 14581461
Pericardial mesotheliomas, 1491
Pericardial metastasis, 1500f
from malignant thymoma, 1459f
Pericardial tamponade, 14381440
Doppler flow velocity records,
14421443
echo-guided pericardiocentesis,
14431444
two-dimensional echo, 14401442
cerebrovascular anatomy, 664
collateral pathways, 669671, 670f
grading carotid stenosis, 679,
682683, 683f
grading internal carotid artery
stenosis, 683684, 684t,
685f688f
intima-media thickness and carotid
plaque assessment, 676679,
678f681f
near occlusion and total occlusion of
ICA, 685688, 690f
posterior circulation, 668669, 670f
scanning protocol, 671676,
672f675f
technical aspects of carotid studies,
671
vertebral arteries, assessment of,
691693, 694f
femoral access complications by,
694701
arterial dissection, 700
AV fistula, 699700, 701f
bleeding and hematoma, 696, 697f
patient-related risk factors, 696
procedure-related risk factors, 696
pseudoaneurysm, 696699, 698f
retroperitoneal bleeding, 696
Perivalvular abscess, 1511
Permanent pacemakers/implantable
cardioverter-defibrillators
complications in, 1212
deleterious effects of, 12171218
echocardiographic findings in,
12101212
endocarditis and, 1214
myocardial perforation, 12151217
2D transesophageal
echocardiography in, 1216, 1216f
reported cases of, 1217t
RT3DE in, 1216f
overview, 1210
RV apical pacing with LV
dyssynchrony, 12171218
thrombosis and stenosis associated
with, 1215
tricuspid regurgitation, 12121214
lead infection associated with,
11141115
time course for, 1213
transthoracic echocardiography for,
12131214, 1213f
Persistent left superior vena cava
(PLSVC), 1958
I-XXIX
I-XXX
pathognomonic LUS sign, 1986
radiography, 1986
Point-of-care diagnosis, 291296
acute care environment and, 294
battery-powered ultrasound imagers
and, 291292, 292f
cardiac disorders, screening and
identification of, 294
abdominal aortic aneurysm, 294295
left ventricular dysfunction, 295
mitral valve prolapse, 295
and future directions, 296
new physical examination
follow-up echocardiography, 293294
ultrasound stethoscope, applications
of, 293, 293f
preparticipation screening of
athletes, 295
remote areas and developing
countries, imaging in, 295
traditional physical examination,
292293, 292f
training requirements, 295296
Poiseuille, Jean, 67
Poiseuilles law, 66, 67
Polycystic ovarian syndrome, in women,
1899
Positron emission tomography (PET),
1429
Posterior circulation, 668669, 670f
Posterior displacement of outlet septum,
1625
Posterior wall thickness (PWTd), 1118
Postsystolic contraction (PSC), 367
Posttreadmill exercise echocardiography,
8
Power Doppler, 113, 114f
advantages and disadvantages of, 72
methodology, 7172
Power Doppler harmonic imaging
(PDHI), 421
Power modulation, 420
Power pulse inversion, 421
PPM. See Patient prosthetic mismatch
(PPM)
Pregnancy
echocardiography in, 19021904
effect of, on HV Doppler, 303
Pre-left ventricular assist device
echocardiogram, 1236t
Premature ventricular contractions, and
HV Doppler, 309, 309f
Primary benign cardiac tumors, 1464
1484
benign cardiac fibromas, 1480
cardiac hemangiomas, 14821484
cardiac lipomas, 14811482, 1483f
cardiac myxoma, 14641475
cardiac rhabdomyoma, 14801481
Primary cardiac lymphoma, 1490
Private tags, 95
Processing technology, TEE, 103, 104f,
105f
Prolonged PR interval
and HV Doppler, 308, 308f
and PV Doppler, 331, 331f
Propagation velocity, 55, 63
in different materials, 56
PROSPECT trial, 369
Prostacyclin (PGI-2), 454
Prosthetic aortic valve abscess,
1109f1110f, 1111f
Prosthetic dehiscence, 1108f1109f
Prosthetic pitch, 1047
Prosthetic regurgitation
paravalvular aortic, 1105f
paravalvular mitral, 1102f1104f
Prosthetic valve dysfunction, 10871092
endocarditis, 10881090
obstruction, 10901092
patient prosthetic mismatch,
10911092
role of 3D TEE in operating room in,
605617, 613f624f
regurgitation, 1087
Prosthetic valve infective endocarditis,
1047
abscess in patient with mechanical,
1047f
dehiscence, 1047
prosthetic pitch, 1047
Prosthetic valves, 1015, 1021f1022f,
10801093, 10921093, 10941112,
19481949
aortic position, failed homograft in,
1087f
aortic prosthetic valve stenosis, 1100
aortic valve and ascending aorta, 1096
assessment of, 10821087
cardiac catheterization, 10921093
cardiac magnetic resonance imaging,
1093, 1093f
cinefluoroscopy, 1092
clinical data for, 1083t
computed tomography scan, 1092,
1092f
3D transesophageal
echocardiography, 11001107
Index
color Doppler reconstruction, 1106f,
1107f1108f
paravalvular aortic prosthetic
regurgitation, 1105f
paravalvular mitral prosthetic
regurgitation, 1102f1104f
prosthetic dehiscence, 1108f1109f
right atrial lipoma, 1096f
3D transthoracic echocardiography,
10951100
of homograft aortic prosthesis, 1100f,
1101f
of St. Jude aortic prosthesis, 1097f,
1101f
of St. Jude mitral prosthesis, 1097f,
1098f1099f
of tissue mitral prosthesis, 1100f
3D visualization, 10941095
2D visualization, 1111t
echocardiographic evaluation of,
1084t
effective orifice area of, 10841087
in elderly, 19481949, 1949f
endocarditis of, 10991100
identification of thrombus, 1098
mitral, 1095
overview, 1080, 1094
paravalvular regurgitation, 1099
prosthetic valve dysfunction,
10871092
endocarditis, 10881090, 1089f
obstruction, 10901092
patient prosthetic mismatch,
10911092
regurgitation, 1087, 1088f
Ross procedure, 1086f, 1090f
surgical mitral and aortic clocks, 1096
types of, 10801082
bioprosthetic valves, 10811082,
1081t, 1082t, 1088f
mechanical valves, 1082, 1083t
true biological valves, 10801081
valved canduit, 1085f1086f
Proximal isovelocity surface area (PISA)
method, 82, 785, 992
for AR severity, 938939
in mitral regurgitation, 887888
MR severity and, 870871
Proximal transverse aortic arch, pulse
Doppler across, 1536f
Pseudoaneurysms
in aortic annulus, 1046f
aortic valve, 1046f
femoral, 696699, 698f
characteristics, 1985
ultrasonic window, 1985
Pulmonary flow acceleration time (PAcT),
1270
Pulmonary hypertension (PH), 171, 890,
10631079
clinical classification of, 1064t1065t
conventional echocardiography,
10631070
2d echocardiographic features, 1067
pulmonary hemodynamics, 1063
1067
stress echocardiography, 10671070
transesophageal echocardiography,
1067
2d echocardiographic signs, indirect,
1069t
diagnosis of
echocardiography role in, 1075f
ESC guidelines for, 1067
diagnostic algorithm in, 10731074
Doppler echocardiographic indices
of, 1065t
hemodynamic definitions of, 1064t
and HV Doppler, 311, 313, 313f
mitral stenosis and, 836838
mitral valve echocardiographic
scoring system, 836
M-mode echo for, 7, 7f
nonconventional echocardiography,
10701073
real time, 3d echocardiography, 1073
strain imaging, 10721073
tissue Doppler imaging, 10701072
overview, 1063
RT 3DE of, 1170
RV systolic function in, impaired,
1069t, 1070f
velocity vector imaging in, 399
Wilkins scoring system, 836837
in women, 19011092
severe, 1903f
Pulmonary interstitial edema, 1984, 1985
diagnosis, 1984
American College of Cardiology
guidelines, 1984
B-lines, 1984
chest X-ray, 1984
chronic heart failure (HF), 1984
gray zone, 1984
limitations, 1984
pulmonary capillary wedge pressure,
1984
prognosis, 1985
I-XXXI
B-lines significance, 1985
heart failure hospitalization, 1985
persistent hemodynamic congestion,
1985
treatment, 1984, 1985
B-lines, 1985
degree of dyspnea, related to, 1985
pharmacological therapy, 1985
tailoring, 1985
pulmonary congestion, reducing of,
1984
medicines effectiveness assessment,
1984
monitoring body weight, 1984
ventricular dysfunction, 1985
Pulmonary regurgitation, 1036, 1038f,
1039f
causes of, 1037f
severe, 1037f
Pulmonary stenosis, 171, 10321036
acquired causes of, 1033f
congenital causes of, 1032f
echocardiographic assessment of,
10341036, 1034f, 1035f1036f
continuous wave Doppler signal in,
1034f
enlarged systolic frame, 1036f
midtransesophageal view, 1036f
patient with tetralogy of fallout, 1035f
transpulmonary valve velocity, 1035f
etiology, 1032
grades of, 1034
normal pulmonary valve area in,
1034
pathology, 1032
pulmonary atresia, 1033
secondary anatomic changes in, 1033
types of, 1033
Pulmonary thrombo disease, 1976
arotic dissection, 1976f
dilatation, 1976
echocardiographic evaluation, 1976
Echogenic structure, 1977f
false lumen, 1977f
post diagnosis, 1976
pulmonary hypertension, 1977f
pulmonary thrombo-embolic, 1976
pulmonary thromboembolism, 1976,
1976f
right ventricle/left ventricle end diastolic dimension, 1976
true lumen, 1977f
ventricular dysfunction, 1976
Pulmonary valve, 10311041
I-XXXII
in adults, 18161818
cardiac catheterization, 1817
MRI/CTA for, 1817
postoperative adult, 1818
surgical treatment for, 18171818
cardiac CT scans, 1040
cardiac MRI, 1040
catheterization, 1040
congenital diseases in newborns,
incidence of, 1032t
echocardiographic evaluation,
10371038
effective regurgitant orifice area, 1038
epidemiology, 10311032
parasternal short-axis view of, 1034f
postpulmonary valve surgery,
10391040
allograft replacement, 1040
pulmonary regurgitation, 1036, 1038f
causes of, 1037f
severe, 1037f
pulmonary stenosis, 10321036
acquired causes of, 1033f
congenital causes of, 1032f
echocardiographic assessment of,
10341036, 1034f, 1035f1036f
etiology, 1032
grades of, 1034
normal pulmonary valve area in,
1034
pathology, 1032
pulmonary atresia, 1033
secondary anatomic changes in, 1033
types of, 1033
pulse Doppler across, 1536f
Ross procedure, 10381039
artificial conduits in, 1039
pulmonary homograft, 1038, 1039f
right ventricular outflow tract, 1038
Pulmonary valve fibroelastoma, 1478f
Pulmonary vascular resistance (PVR),
1063, 1065t, 1066, 12721273
Pulmonary vein ablation
cardiac computed tomography for,
20542057
computed tomography for, 2054
2057
Pulmonary vein isolation, for atrial
fibrillation, 566, 568569, 569f,
570f
Pulmonary veins (PV), 16701684, 1963
ablative therapies, 1963
abnormalities, 1963
additional ablation, 1963
anatomy, 1963
delineating, 1963
anatomy of, 325
anomalies of, 16721673
abnormal number of pulmonary
veins, 1672
anomalous pulmonary venous
return, 16721673
blood flow in
aging and, 329330, 330f
factors affecting, 329331
loading conditions and, 330331
physiology of, 325327, 326, 328f
respiration and, 329
cardiac MR, 1963
color contrast in, 1552f
color Doppler of, 1533f
CT angiography, 1963
disease states, and PV blood flow
atrial fibrillation, 333, 333f
atrial flutter, 333, 333f
atrial septal defect (ASD), 342, 342f
atrioventricular dissociation,
331332, 332f
cardiac tamponade, 341
conduction disorders, 331
constrictive pericarditis, 340341,
340f, 341f
junctional rhythm, 333, 334f
left ventricular end-diastolic
pressure, assessment of,
334335, 334f, 335f
mean left atrial pressure, assessment
of, 335, 335f
mitral regurgitation, 336338, 337f,
338f
mitral stenosis, 338339, 339f
myocardial relaxation pattern,
impaired, 335336
paced rhythm, 334, 334f
premature ventricular contractions,
332, 332f
prolonged PR interval, 331, 331f
pseudonormal filling pattern, 336
pulmonary vein stenosis, 342, 342f
rate and rhythm disorders, 332334
restrictive cardiomyopathy, 339340,
340f
restrictive filling pattern, 336, 337f
short PR interval, 331, 331f
sinus bradycardia, 332, 333f
sinus tachycardia, 332, 332f
flaring degree, 1964
floating thrombus, 1965f
Index
flow pattern, 1964
atrial reversal, 1964
diastolic dysfunction, 1964
diastolic forward flow, 1964
mitral regurgitation, 1964
systolic forward flow, 1964
A Flutter, 1963
imaging, 1963
imaging of, 325329
technical considerations in, 327, 329,
329t
transesophageal echocardiography,
327
transthoracic echocardiography, 327
isolation, 1963
left inferior, 1963
left upper, 1963f
limitations and technical pitfalls, 342,
342f
localization, 1964
lower, 1963f
macro-reentrant tachycardia, 1963
midesophageal, 1963
level, 1963f
probe, 1963
morphological features, 1963
normal flow pattern of, 16701672
ostium, 1964
peak diastolic velocity, 1965f
pulmonary hypertension, 1964
pulse Doppler of, 1533f
right middle, 1965f
to right-sided left atrium, 1546f
right upper, 1963f
caval view, 1964
spectral Doppler of, 325345
artifacts, 343345, 344f345f
differential diagnosis for abnormal
PV flow patterns, 345, 345f
limitations and technical pitfalls,
342343, 344f
stenosis, 1965f
development, 1964
systemic venous anomalies, 1678
1684
systolic forward flow, 1964
thoracic aorta,descending, 1963
total anomalous pulmonary
venous connection, 16731678,
1673f, 1674f1675f
anomalous drainage of, 1676
diagnosis steps in, 16731676
echocardiographic goals, 1673
features of, 1673
pulmonary vein stenosis and, 1678
pulmonary venous confluence,
16731675
septum primum malposition defect
and, 16761678, 1677f
Pulmonary vein stenosis
and PV Doppler, 342, 342f
with TAPVC, 1678
Pulmonary venous confluence (PVC),
16731675, 1674f
orientation and site of drainage of,
16741675
Pulmonary venous flow, MR severity and,
869
Pulmonic valve
3D echo of, 522523, 522f, 523f
ventricular assist devices and, 1232
Pulmonic valve disease, 3DE assessment
of, 284, 284f, 285f
Pulsed Doppler, 6364. See also Spectral
Doppler
Pulsed wave Doppler
advantages and disadvantages of, 69
and aliasing, 70, 70f
methodology, 6970, 69f
Pulse inversion, 420
Pulse pressure (PP), 467
Pulse repetition frequency (PRF), 63, 69,
736
Pulse wave velocity (PWV) analysis,
466467, 466f, 467f
PVR. See Pulmonary vascular resistance
(PVR)
PWTd. See Posterior wall thickness
(PWTd)
Pyramidal imaging, 241
PZT (lead zirconate titanate) ceramics,
102, 103f
Q
QLab, 385,514
Quadrature detector, 66
Quadricuspid aortic valve, 1620, 1620f
in female, 1915f
Quantification techniques in
echocardiography, noninvasive,
705729
aortic valve assessment, 727728,
728f
3DE quantification tools, clinical
applications of, 721723
left ventricle global and regional
function, 722
left ventricle mass, 722, 722f
left ventricle shape analysis, 722723,
723f
fluid mechanics algorithms and
particle imaging, 720721, 721f
future outlook, 728729, 729f
global strain, 712714, 712f715f
goal of, 706
heart chamber segmentation
algorithms, 706707, 707f
limitations of speckle tracking
echocardiography, 720
mitral valve assessment, 725727,
726f728f
parametric display, 708711,
709f711f
regional strain, 714715, 715f, 716f
right ventricular quantification,
723725, 723f726f
rotation, twist, and torsion, 717720,
719f
segmental/regional analysis of left
ventricle, 707708, 708f, 709f
strain quantification and speckle
tracking algorithms, 711712
three-dimensional speckle tracking
and strain, 715, 717, 717f719f
Quantitative gated single-photon
emission computed tomography
(QGSPECT), 271
R
RABT (red away blue toward), 64
Radial strain, 362, 363f, 389
Range ambiguity, 736. See also Artifacts
RAP. See Right atrial pressure (RAP)
Rashkind procedure, 531
RBBB. See Right bundle branch block
(RBBB)
RCM. See Restrictive cardiomyopathy
(RCM)
Reactive hyperemic index (RHI), 467468
Reactive oxygen species (ROS), 454
Real time, three-dimensional TEE
(RT3DTEE) probe, 101102, 101f,
102f
Real time 3D echo (RT3DE), 11421143
Real time (RT) 3D mode, 269
Real time (RT) 3D TTE, 74
Real time/live 3D echocardiography, 241
Real time myocardial perfusion
echocardiography (RTMPE),
444445
I-XXXIII
Index
disc summation method, 1169f
of pulmonary hypertension, 1170
second-generation, 11671168
transverse shortening and, 1166
Right ventricle, evaluation of, 11341148
apex-forming, 1136
cardiac magnetic resonance imaging,
1136f, 11441145, 1144f
cardiovascular computed
tomography, 1145
contractile function, 11401141
Doppler echocardiography, 1139
1141
continuous wave Doppler spectral
display, 1139
conventional doppler, 11391140
pulsed wave Doppler spectral
display, 1140, 1142f
tissue doppler, 11401141, 1141f
echocardiography, 1136
M-mode, 11361138
three-dimensional, 11421143
transesophageal, 1143
two-dimensional, 11381139, 1139f,
1140f, 1141f
echo windows for, 1137f
gadolinium contrast in, 1145
geometry of, 1135f
hemodynamics, 11431144
HV systolic filling fraction, 1144
right atrial pressure, 11431144
morphology, 11351136
regional RV wall motion, 1136
right ventricular outflow tract, 1135
RV systolic function, 11351136
myofibrillar arrangement of, 1136
1137
overview, 11341135
evaluation of, 11341148
real time 3D echo, 11421143
Simpsons method of discs for, 1140f
triangle of dysplasia, 1135, 1135f
two-dimensional strain (speckle
tracking), 11411142, 1142f
volume/body surface area, 1143t
Right ventricle fractional area change
(RVFAC), 1071f
Right ventricular diastolic dysfunction,
1644f
and HV Doppler, 313315, 315f
Right ventricular dysfunction
ICM/NICM and, 1422
mild residual, 1242f
S
Sacrococcygeal teratoma, 1530
Saline contrast, 1213
Saline contrast chocardiography, 435,
436f
SAM. See Systolic anterior motion (SAM)
Sapien valve, balloon expandable,
541543
Sarcoidosis, 1405, 1405f, 18761879
Sarcomas, 14841488, 2054
right ventricular, 1487f
SAVR. See Surgical aortic valve
replacement (SAVR)
Scan line, 77
Scanners, trends, 19911993
acoustic structure quantification,
liver, 1992, 1992f
advantage, 1992
B-mode signal
analysis, 1992
cable-free transducer systems, 1992
development of, 1992
capacitance micro-machined
ultrasound transducer, 1992
complete cross-sectional display
computed tomography, 1993
magnetic resonance, advantages of,
1993
contrast-enhanced ultrasound, 1991,
1992
crystal piezoelectric materials, 1991
electrostatic loudspeaker, 1992
fusion imaging, 1993f
Lead zirconate titrate, 1992
miniaturization, 1991
scanner user interfaces, 1993
semiconductors, 1992
smartphone ultrasound system, 1991,
1991f
I-XXXV
software-driven scanners, 1991
symmetrical effect, 1992
tissue characterization conference,
1992
transesophageal echocardiography
live/real time three/four dimensional, 1992, 1992f
ultrasound system, 1991
SCD. See Sudden cardiac death (SCD)
Sclerosing mediastinitis, 1515f1517f
Screen, 60
Secondary cardiac tumors, 14921500
carcinoid heart disease, 1500
malignant melanoma, 1500
Sector angle, and frame rate, 59, 60f
Sector probe, 58, 58f
Secundum ASD, 17341742
device embolization in, 1739
3D TEE in, 17381739, 1739f,
1740f1742f
repair of, 17341736
Semilunar valves, short axis of, 1536f
Septal aneurysms, 182
Septal wall thickness at diastole (SWTd),
1118
Septic cardiomyopathy, 14051407, 1406f
echocardiographic features of,
11406407
Septum (ventricular), 11941198, 1205f
echocardiographic pattern of, 1196f
endocardial and epicardial fibers,
1195f
fiber orientation of, 1195f
septal line, 1194
ultrasonic crystal tracings, 1197f
Septum primum malposition defect
TAPVC and, 16761678, 1677f
Serial evaluation, of patients with 3DE,
11641165
Shah, Pravin, 11
Shear rate, 452
Shear stress, 452454, 452f454f, 455. See
also Endothelial dysfunction
Short PR interval
and HV Doppler, 307, 308f
and PV Doppler, 331, 331f
Short-tau-inversion-recovery (STIR),
2020
Short-term circulatory support devices,
12261227
Abiomed AB5000, 1227
Impella catheter-based assist device,
1227
TandemHeart system, 1227
muscular, 1804
postoperative adult, 1805
supracristal, 1803
venturi effect, 1803f
Shunt lesions/septal defects, 17331747
atrial septal defects, 1734
atrioventricular septal defectsts,
17461747
secundum ASD, 17341742
sinus venosus ASD, 17421743
unroofed coronary sinus, 1743
ventricular septal defects, 17431746
Sickle cell disease, three-dimensional
speckle tracking and, 376t
Side lobe artifacts, 6263, 63f, 736. See
also Artifacts
transesophageal echocardiography
and, 107, 108f
Sigmoid septum, of elderly, 167
Single element transducers, 76, 76f
Single-photon emission computed
tomography (SPECT), 14251427
Single plane transesophageal echo (TEE)
probe, 99, 99f
Single ventricle, velocity vector imaging
in, 392394
Sinotubular (ST) junction, 168, 1621
Sinus bradycardia
and HV Doppler, 306, 307f
and PV Doppler, 332, 333f
Sinus of Valsalva aneurysm, 16301632,
1631f, 1784
in adults, 18111812
aortic regurgitation and, 1631
associated anomalies, 16311632
echocardiographic evaluation of,
16301631
hypoplastic left heart syndrome, 1784
right ventricular outflow obstruction
and, 1784
ruptured right, 1784f1785f
VSD and, 1631
Sinus tachycardia
and HV Doppler, 306, 307f
and PV Doppler, 332, 332f
Sinus venosus ASD (SVASD), 1586,
17421743, 1743f
Sinus venous defect, 1800
Sjogrens syndrome, 1545
SLE. See Systemic lupus erythematosus
(SLE)
Slicing methods, 79
Smearing, 59
Society of Cardiovascular
Anesthesiologists (SCA), 638
Index
high frame rate, 89
imaging mode, 90, 90f
lateral gain, 8990
raw data format, 90
scan range, 89
two-dimensional frequency, 90
Speckle-tracking echocardiography
(STE), 360376, 382, 13021304.
See also Velocity vector imaging
(VVI)
cardiac muscular anatomy, 360361,
361f
image acquisition and processing,
367
limitations of, 374 375
myocardial infarction, 1302
myocardial ischemia, 1302
myocardial viability, 13021303
reperfusion, 1302
strain and, 362365, 362t, 363f
three-dimensional, 372373, 373f,
374f
area strain measurement and, 373,
375, 375f, 375t
clinical applications of, 373374, 376t
and tissue Doppler imaging, 360, 361t
two-dimensional, 365367, 365t, 366t
cardiac transplantation and, 371
cardiomyopathies and, 369371
chemotherapy cardiotoxicity and,
371372
clinical application of, 367372, 368t
congenital heart disease and, 372
coronary artery disease and, 367
CRT for heart failure and, 369
right ventricular function and, 372
rotational dynamics, role of, 372, 372f
valvular heart diseases and, 371
SPECT. See Single-photon emission
computed tomography (SPECT)
Spectral broadening, 68
Spectral Doppler, 66, 112113, 113f
transthoracic echocardiogram,
136137, 137f
Splenic syndromes, 1704
Squatting stress echocardiography,
13231327, 13241326
acute LV remodeling on, 1325
advantages of, 1326
chronic obstructive pulmonary
disease, 1323
coronary artery disease, 1323
electrocardiography (ECG), 1323
end-systolic frames in, 1324f
limitations of, 1326
LV function and, 1324f
mechanism of, 1325
overview, 1323
protocol, 1324
results/observations, 13241325
vs. dobutamine stress
echocardiography, 1325, 1326t
wall motion abnormalities, 1323
left ventricular, 13241325
mechanism of, 1325
St. Jude aortic valve, 1085f
St. Jude bileaflet mitral valve, 1092f
Stanfard classification, 1974
apical, 1976t
ascending aorta, 1974
diastolic collapse, 1976
dissections, 1974
esophagus, 1976
left parasternal, 1976t
lumen, 1976
mitral valve, 1975f
pressure Half-time, 1975t
systolic expansion, 1976
transesophageal probe, 1976
transthoracic views, 1976t
visualization, 1974
Stanford type A aortic dissection, 935,
936f
Staphylococcus aureus, 1048
STARFlex occluder, 557
StarrEdward valve, 1085f
State Food and Drug Administration
(SFDA),China, 110
State-of-the-art, 11801181
composite of, 11811183
HVMB model, 1181
muscle contraction, asynchronous,
1181
Statins, 450
Steady-state free precession (SSFP), 2020
for aortic regurgitation, 2010
Stenosis of pulmonary outflow, 1647
1648
aortic outflow obstruction, 1647
remote ventricular septal defect, 1648
ventricular septal defect, restriction
of, 1647f, 1648
Stenotic aortic valve, morphology,
16191620
STIC. See Spatiotemporal image
correlation (STIC)
Stiffness index (SI), 465, 467
I-XXXVII
Strain
calculation of, 362
imaging, 360
normal, 385, 389
normal rotation and torsion values,
364t
normal values for, in normal adults,
364t
peak, 365
physics of, 385, 389, 389f
rate, 362
shear, 385, 389
types of, 362, 362t, 363f
Streptococcus viridans, 1054
Stress (Takotsubo) cardiomyopathy
three-dimensional speckle tracking
and, 376t
Stress echocardiography, 226227,
13061322
cardiac event rate as function of,
1315f
cost-effectiveness of, 13171318
Doppler hemodynamics with,
13181319
aortic valve disease, 1318
dynamic pulmonary hypertension,
13181319, 1319f
hypertrophic cardiomyopathy, 1318
latent diastolic dysfunction, 1318
mitral valve disease, 1318
fundamentals of, 13061307
future directions for, 1319
impact on patient outcome, 1317
interpretation of, 13091319
diagnostic accuracy to detect CAD,
1310, 1313t
transient ischemic LV cavity
dilatation, 1315
warranty time, 1317
myocardial infarction vs. cardiac
death by, prediction of,
13111315
myocardial perfusion and, 1319
overview, 1306
predictors of risk, 1314t
prognostic value of, 1316t
risk stratification and prognosis,
13101315, 1314f
left atrial size in, role of, 1317
myocardial wall motion abnormality,
1311
RV wall motion abnormalities in, role
of, 1315
safety of, 13081309
membranous subaortic stenosis,
1625
posterior displacement of outlet
septum in, 1625
Subvalvular infundibular stenosis, 1033,
1035f, 1036f
Sudden cardiac death (SCD), 1348
Summed difference score (SDS), 1158
vs. WMS difference, 1159f, 1159t
Summing, 77
Superior vena cava (SVC), 1140
anomalies of, 16781682
absent right, 1680
aneurysm of, 1682
clinical significance of, 16801681
defect in wall of coronary sinus,
16791680
left superior vena cava to coronary
sinus, 16791680
left superior vena cava to left atrium,
1680
persistent left, 16781679
right superior vena cava to left
atrium, 1681, 1681f
heterotaxy syndrome and, 1705
normal spectral Doppler of, 304, 304f
Supraclavicular approach, of three dimensional echocardiography,
244, 258f259f
Suprasternal approach, of three dimensional echocardiography,
244, 257f
Supravalvular aortic stenosis, 16261628,
1626f
in adults, 18251826
aortic valve anomalies, 1627
branch pulmonary arteries in, 1628,
1628f
coronary artery abnormalities, 1628
morphology, 1627
Supravalvular stenosis, 1033
Supraventricular tachycardia (SVT), 1542,
1957
Surface rendering, 79, 79f
Surgical aortic valve replacement (SAVR),
540541
in aortic stenosis, 926927
in LFLG-AS with low ejection
fraction, 927
PLFLG-AS and, 922
in PLFLG-AS with normal ejection
fraction, 927
Suture-less valves, 606
SVC. See Superior vena cava (SVC)
Index
total anomalous systemic venous
drainage, 1684
venous valves, 16831684
Systolic abnormalities, of tricuspid valve,
986
Systolic anterior motion (SAM), 1348
echocardiographic evaluation of,
13511352, 1352f
mitral-septal contact, 1359f
of mitral valve, 13501354, 1350f,
1359f
pathophysiology of, 1353
Systolic blood pressure (BP),
pseudonormalization of, 911
Systolic dysfunction, and HCM, 1356
1358
Systolic dyssynchrony index (SDI), 373,
1726
Systolic function
in dilated cardiomyopathy, 1371
in ICM/NICM, 1419
Systolic myocardial dysfunction, 1131f
Systolic pulmonary artery pressure
(SPAP), 1063, 1065t, 1066, 1067,
1270, 1270f, 1271f
T
TAA. See Thoracic aortic aneurysm (TAA)
Tachycardia-induced cardiomyopathy,
1388, 1395f
Takotsubo cardiomyopathy, 1388, 1394f
in women, 18991900
vector velocity imaging, 1900
TandemHeart device, 1225f, 1227,
12501251
TAPSE. See Tricuspid annular plane
systolic excursion (TAPSE)
in peripartum cardiomyopathy, 1381
TAPVC. See Total anomalous pulmonary
venous connection (TAPVC)
TAPVR. See Total anomalous pulmonary
venous return (TAPVR)
TaussigBing anomaly, 1647
Tei's index, 1120, 1120f, 1419
Temporal tap, 674, 675f
Tenting area, 1422, 1423f
Tetralogy of fallot (TOF), 1763
with absent pulmonary valve, 1637,
1637f
in adults, 18291835
cardiac catheterization, 18341835
echocardiography, 18301832
MRI/CT for, 1835
postoperative adult, 1835
postoperative adult, surgery in, 1835
stress echocardiography, 18321834
aortic regurgitation in, 1641, 1641f
cardiac catheterization, indications
for, 1641
computed tomography for, 2062,
2063f
echocardiographic measurements in,
16401641, 1640f
Hoffman's variant, 1635f, 1636f
postoperative evaluation of, 1641
1644, 1642f
with pulmonary stenosis, 1633
velocity vector imaging in, 391392
Thebesian valve, 1503
Thermal index (TI), 111
Thoracic aortic aneurysm (TAA),
19341937
in Marfan syndrome, 1936
natural history of, factors impacts,
1937
Thoracic cage artifacts, 433434, 434f
Thoratec CentriMag system, 1127
Thoratec HeartMate II, 1223f
Thoratec paracorporeal ventricular assist
device, 1227
Three-dimensional echocardiographic
guidance of percutaneous
procedures, 531571. See
also Catheter-based
transcutaneous interventional
procedures
Three-dimensional echocardiography
(3DE), 1418, 240267, 705
advantages/disadvantages of, 262,
267
apical approach, 244, 254f, 255f
artifacts in, 736737. See also
Artifacts
basics of, 7485
beam forming, 7778
color Doppler imaging, 248, 254255,
266f
evolution of, 7475, 75f
examination protocol, 241244,
242f243f
image quality, limitations in
aperture, 80
artifacts, 8081
gating, 80
spatial and temporal resolution, 80,
80f
left parasternal approach, 244,
245f254f
I-XXXIX
multibeat acquisition in, 7475, 75f
in operating room, 577634
aortic valve disease, 582588
cardiac masses, 617627
limitations of 3D TEE and future
directions, 628629
mitral valve disease, 577582
native valve endocarditis, 597604
prosthetic valve dysfunction, 605617
tricuspid valve disease, 589590,
593597
quantification, 8185
left ventricular, 8285, 84f
mitral valve, 8182, 82f, 83f
rendering in, 78, 79f
2D tomographic slices, 7980
surface rendering, 79
volume rendering, 7879, 79f
right parasternal approach, 246, 248,
259f266f
strengths of, 81
subcostal approach, 244, 256f255f
supraclavicular approach, 244,
258f259f
suprasternal approach, 244, 257f
technology related to, 240241,
240f241f
transducer technology and, 76
3D matrix array transducers, 7677,
77f78f
linear/phase array transducers, 76,
76f
single element transducers, 76, 76f
for valvular heart disease, 515528
aortic valve, 520522, 520f
case examples of, 525528
data acquisition, 515516, 516f, 517f
image optimization, 516
mitral valve, 516520, 517f, 518f
pulmonic valve, 522523, 522f, 523f
tricuspid valve, 523525, 524f
Three-dimensional epiaortic
ultrasonography, 641
Three-dimensional intracardiac
echocardiography, 652, 653f
Three-dimensional matrix array
transducers, 7677
Three-dimensional (3D) speckle tracking,
92, 94f95f
frequency, 95
modes, 96, 97f
for morphological study, 94f
one-beat acquisition, 96
raw data storage, 95
I-XL
scan range/volume width, 96
triggered full volume, 96, 97f
volume rate, 96
wall motion tracking, 92, 95, 97f
Three-dimensional speckle tracking and
strain, 715, 717, 717f719f
Three-dimensional speckle tracking
echocardiography (3DSTE),
372373, 373f, 374f, 17271728,
1727f
area strain measurement and, 373,
375, 375f, 375T
clinical applications of, 373374, 376t
use of, 404405, 405f
Three-dimensional stress
echocardiography, 13281336
advantages of, 13291330, 1330t
contraction front mapping in, role of,
13341335
contrast in, 1335
current standards vs., 13311334
adenosine stress test, 13331334
dipyridamole stress test, 1334
dobutamine stress test, 13311333
treadmill exercise stress test, 1333
future directions, 1335
image acquisition in, 13301331
overview, 1328
parametric imaging in, 1334
postacquisition analysis, 1331
stress protocol in, 1331
three-dimensional transducers, 1329,
1329f
vs. 2DSE, in wall visualization, 1334
Three-dimensional transducers, 1329
Three-dimensional transesophageal
echocardiography (3D TEE), 18,
507514
aortic valve display in short-axis view
by, 511f512f
biplane (X-plane) image of left atrial
appendage, 509f
image display recommendations,
511, 511t
image optimization, 510
colors, 510
compression, 510
cropping and rotation, 510
gain, 510
smoothing, 510
imaging modalities in, 509t
full volume, 509
full-volume and live 3D color flow,
509
real time (RT) 3D, 508
simultaneous biplane mode, 509
zoom view, 508
imaging protocol for, 510t
indications for, 508t
procedure for
image acquisition, 508509
imaging sequence, 509510
preprocedural planning, 508
RT3D TEE imaging, advances in,
511512
technology for, 507
uses of, 512
catheter-based LAA closure, 512
congenital heart disease, 513
LAA clot, detection of, 512
left ventricular function and
dyssynchrony assessment, 514
mitral prosthesis paravalvular leak
closure, 512
mitral stenosis and balloon
valvotomy, 513514
mitral valve assessment, 513
transcatheter aortic valve
replacement, 512513
trans-septal puncture, 512
Three-dimensional transthoracic
echocardiography examination,
268286
aortic annulus, 280
aortic regurgitation, 280
data acquisition, methods for
cropping, 269270
3DE color flow Doppler imaging, 269
image display, 269270
multiplane mode, 268269
multiple-beat 3DE imaging, 269
real time 3DE, 269
tomographic slices, 270, 270f
left ventricular assessment
image acquisition methods, 270, 270f
LV regional function, 271272
normal values, 272
volume and systolic function
assessment, 271
mitral regurgitation, 282283, 282f,
283f
mitral stenosis, 280282, 281f
mitral valve assessment, 280, 281f
pediatric and fetal cardiac
pathologies and, 285286
pulmonic valve disease, 284, 284f,
285f
regional LV function, 276
Index
aortic stenosis, 279
aortic valve assessment, 278f, 279,
279f
contractile reserve, 276
left and right atria, 276277, 277f
left ventricle twist, 276
right ventricle, 277278
valvular assessment, 278, 278f
reproducibility, 272273
3D speckle-tracking applications,
275, 275f
3D stress echocardiography, 274275,
274f
global LV function, evaluation of, 276
LV mass, 273274, 273f
methods of validation, 275276
tricuspid valve disease, 283284, 283f,
284f
Three-dimensional TTE, 159161, 161f
Thyroid disorders, 18791880
T2* (star) imaging, 2020
Timeacoustic intensity curve, 442
Time gain compensation (TGC), 61
Tissue Doppler (TD) imaging, 14, 64, 72,
349357, 360, 382
in atrial fibrillation, 353
color TD imaging, 349
development of, 350
diastolic function, assessment of,
352353, 355f, 356f
for LV dyssynchrony, 353, 354f
LV filling pressures, estimation of,
352353
myocardial disease and, 350352
and prognosis, 354355
RV function, assessment of, 354,
356f357f
spectral TD imaging, 349
and technical considerations,
349350, 350f
transthoracic echocardiogram, 137
uses of, 350357
Tissue plasminogen activator (tPA), 1996
Tissue velocity imaging. See Tissue
Doppler
TomTec Cardiac Performance Analysis,
380
Torsion (cardiac motion)
with co-contraction of base and helix,
1190f
compression, 1188
definitions, 11831184
during ejection, 1191f
left ventricle, 11861188, 1186f
longitudinal shortening, 1188
structural reasons for, 1184f
and untwisting, 1200
Total anomalous pulmonary venous
connection (TAPVC), 1577, 1672,
16731678, 1673f, 1674f1675f. See
also Pulmonary veins
anomalous drainage of, 1676
diagnosis steps in, 16731676
echocardiographic goals, 1673
features of, 1673
infracardiac, 1674
pulmonary vein stenosis and, 1678
pulmonary venous confluence,
16731675
septum primum malposition defect
and, 16761678, 1677f
Total anomalous pulmonary venous
return (TAPVR), 1743
Toxic cardiomyopathies, 13961397
adriamycin-induced
cardiomyopathy, 1397f
alcohol-induced cardiomyopathy,
1397
chemotherapy-induced
cardiomyopathy, 13961397
Training, in echocardiography, 750751,
759f, 760f
appropriate use criteria, 758
cardiac sonographers, training of,
753754
certification and maintenance of
proficiency, 758, 759f, 760f
contrast echocardiography and, 757
CT and MRI, training in, 755
duration and sites, 755
fellowship training, 751752, 752t
content of, 754755
level 1 training, 751
level 2 training, 751
level 3 training, 752
intraoperative TEE and, 756757
intravascular and intracardiac
ultrasound and, 757
noncardiologists, training of, 752753
in pediatric echocardiography and
congenital heart disease, 753
special echocardiographic
procedures and, 755
stress echocardiography and, 757
three- and four-dimensional TTE and
TEE and, 757
tissue Doppler and speckle tracking
echocardiography, 757758
training requirements, 751t
transesophageal echocardiography
and, 755, 756t
Transcatheter aortic valve implantation
(TAVI). See Transcatheter aortic
valve replacement (TAVR)
Transcatheter aortic valve replacement
(TAVR), 512513, 540546
AVA calculation, 543
CTA for, 2058f
CT and, 2029t, 20572059
in elderly, 1935f
intra- and postprocedural monitoring
by 2D/3D TEE, 543, 546, 546f
patient seletion for, 543
TAVR valves, 541543, 543f
three-dimensional speckle tracking and,
376t
Transducer probe, 60
Transducers, 58
Transducer technology, 102, 103f
Transesophageal echocardiography
(TEE), 13, 99116, 487
acoustic shadowing, 61, 62f
artifacts
reverberation, 107, 109110, 109f
side lobes, 107, 108f
sound velocity, effect of, 107, 107f,
107t, 108t
of coronary arteries, 13381340
current/future technologies, 112116
and artifacts, 113116, 114f
CMUT technique, 115f, 116, 116f
color Doppler, 113, 113f, 114f
power Doppler, 113, 114f
spectral Doppler, 112113, 113f
image quality
aperture and, 104, 106f
focus and, 106, 106f
frequency and, 104105, 105f
kinds of
dual plane probe, 99100, 100f
matrix array probe, 101102, 101f
miniaturization technology, 102103,
103f, 104f
processing technology, 103, 104f, 105f
real time, three-dimensional TEE,
102
rotary plane probe, 100, 100f, 101f
single plane probe, 99, 99f
transducer technology, 102, 103f
variable plane probe, 100, 100f
pulmonary veins, imaging of, 327
safety considerations, 110
I-XLI
acoustic energy safety, 111
electric safety, 110111
electromagnetic compatibility, 112
heating safety, 111
mechanical safety, 111112
three-chamber view, 60f
training in, 755, 756t
Transesophageal echocardiography,
tricuspid valve
in inferior myocardial infarction,
1014f
three-dimensional, 988990
two-dimensional, 988, 988f, 989f990f
bicaval view, 988f
longitudinal plane examination, 989f
mid-esophageal four-chamber view,
988f
normal tricuspid valve anatomy,
989f990f
Transjugular intrahepatic portosystemic
shunt (TIPS), 319
Transmitral diastolic inflow, 1126f
Transmitter, 60
Transposition of great arteries (TGA),
16531663, 17541763
anatomy, 16531654, 1653f
associated defects, 16571661
coronary arteries, 1661
fixed anatomical obstruction,
16601661
inlet VSD, 1659
left ventricular outflow obstruction,
16591661
septal defects, 16571659
VSD, 16581659
associated lesions, 1653
computed tomography for, 2059
2062, 2061f
coronary artery patterns in, 1661
1663
intramural coronary, 16621663
inverted origin of coronaries, 1662
single left coronary artery, 1662,
1662f1663f
single right coronary artery, 1661
echocardiographic evaluation,
16541656
chamber size, 16551656, 1656f
two-dimensional, 16541655, 1654f,
1655f
Transseptal cardiac catheterization, 233
Transseptal puncture, 512, 532533, 534f
Transthoracic echocardiography (TTE),
132162
I-XLII
apical window, 146, 148
five-chamber plane, 153154, 153f,
154f, 154t
four-chamber plane, 148153,
148f153f, 149t
long-axis or three-chamber plane,
155, 156f
two-chamber plane, 154155, 154f,
155f
beginning of, 132, 135
cardiac structures assessed in,
133t134t
apical window, 133t134t
parasternal window, 133t
of coronary arteries, 13371338
four-chamber view, 60f
imaging modalities
color flow Doppler, 137
M-mode echocardiography, 136, 136f
spectral Doppler, 136137, 137f
tissue Doppler imaging, 137
two-dimensional echocardiography,
135136, 135f
imaging windows and planes, 135
left ventricle and wall motion
determination, 161162, 162f
parasternal window
linear measurements, 139141,
139f141f
parasternal long-axis plane, 137139,
138f, 139f, 139t
parasternal short-axis plane, 144146,
145f147f, 145t
right parasternal long axis view,
141142
right ventricle inflow view, 142, 142f,
142t, 143f
right ventricular outflow tract view,
142144, 143f, 143t, 144f
patient positioning for, 135
pulmonary veins, imaging of, 327,
328f
subcostal window
color and spectral Doppler imaging,
158159
four-chamber view, 155, 156f, 156t,
157f
great vessel imaging, 157158, 158f,
159f
short-axis views, 157, 157f
suprasternal notch window, 159, 160f
three-dimensional, 159161, 161f
of tricuspid valve
three-dimensional, 988990
two-dimensional, 986988, 987f
Index
torrential, 996f
transesophageal echocardiography,
1000f1002f
before and after annuloplasty, 1001f
coronary sinus, 1000f
two jets of, 1001f
Tricuspid regurgitation velocity (TRV),
1063, 1066, 1066f, 1067
Tricuspid stenosis, 812813, 10041007
carcinoid heart disease and, 1006
diet drug-induced valvulopathy, 1006
in elderly female with rheumatic
heart disease, 1006f
grading scales, 1004t
hemodynamically significant,
findings, 1004t
and HV Doppler, 311, 312f
Loefflers syndrome, 10061007
severity of, 813
transthoracic echocardiography
three-dimensional, 1006f
two-dimensional, 1005f1006f
Tricuspid valve (TV), 589590, 1233
in adults
cardiac catheterization, 1815
Ebsteins anomaly, 18131814, 1815t
echocardiography, 1814
MRI/CT for, 18141815
postoperative adult, 18151816
stress echocardiography, 1814
tricuspid valve surgery, 1815
anatomy of, 984, 989f990f
congenital anomalies of, 1022,1616
1618
congenital lesions of, 1616t
congenitally unguarded tricuspid
orifice, 16171618, 1617f
Ebsteins anomaly of, 16161617,
1616f
tricuspid valve prolapse, 1617
3DE assessment of, 278, 278f
3D echo of, 523525, 524f
and dilated cardiomyopathy, 1376
dilated cardiomyopathy and, 1376
flail, 10071029
M-mode echocardiography, 984986
Ebsteins anomaly, 986f
functional events, demonstrating,
984f
septal leaflet, identification, 985f
systolic abnormalities, 986f
using contrast injections,
identification, 985f
pacer and, 1215f
pulsed wave Doppler across, 1533f
three leaflets of, 991f
transesophageal examination
three-dimensional, 988990
two-dimensional, 988, 988f, 989f990f
transthoracic examination
three-dimensional, 988990
two-dimensional, 986988, 987f
ventricular assist devices and, 1232,
1233f
Tricuspid valve annulus, 1474f
Tricuspid valve diseases
anatomy of tricuspid valve, 812
3DE assessment of, 283284, 283f,
284f
tricuspid regurgitation, 813816
color flow imaging, 814
continuous wave Doppler, 815, 815f
3D echocardiography, 816
flow convergence method, 814815
measurement of VC, 814
pulmonary artery pressure, 815
pulsed wave Doppler, 815
role of 3D TEE in operating room in,
589590, 593597, 601f608f
RV dimensions and function,
815816
severity of, 816, 816t
transesophageal echocardiography
in, 816
two-dimensional echocardiography,
814
tricuspid stenosis, 812813
severity of, 813
Tricuspid valve endocarditis, 10081015,
1058f
in adult female, 1017f
MRSA positive, 1020f
Tricuspid valve fibroelastoma, 1023f,
1477f, 1479f
Tricuspid valve myxoma, 1465f
Tricuspid valve prolapse, 10071029, 1617
2d transesophageal
echocardiography, 1010f1011f
in elderly male with dyspnea, 1011f
myxomatous degeneration, 1011f
prosthetic valves and, 1015,
1021f1022f
transthoracic echocardiography
three-dimensional, 1011f
two-dimensional, 1009f1010f
TV endocarditis, 10081015
TV tumors, 1015
I-XLIII
I-XLIV
frame rate, 8889, 89f
frequency, 90
gain level, 89, 89f
high frame rate, 89
imaging mode, 90, 90f
lateral gain, 8990
raw data format, 90
scan range, 89
Two-dimensional speckle tracking
echocardiography (2D STE),
365367, 365f, 366f
clinical application of, 367, 368t
cardiac transplantation, 371
cardiomyopathies, 369371
chemotherapy cardiotoxicity,
371372
congenital heart disease, 372
coronary artery disease, 367
CRT for heart failure, 369
right ventricular function, 372
rotational dynamics, role of, 372, 372f
valvular heart diseases, 371
Two-dimensional stress
echocardiography, 13281329
vs. 2DSE, in wall visualization, 1334
Two-dimensional subcostal
echocardiography, abdominal
aorta, 222f
Two-dimensional (2D) TEE examination,
480485
esophageal intubation, 481
informed consent for, 480
patient selection for, 480
preparation and conscious sedation,
480
procedure, 481485, 481f485f
bicaval view, 482f
color Doppler interrogation of aortic
valve in long axis, 483f
color Doppler interrogation of aortic
valve in short axis, 484f
color Doppler interrogation of atrial
septum, 482f
color Doppler interrogation of LAA,
482f
color Doppler interrogation of mitral
valve, 483f
color Doppler interrogation of
tricuspid valve, 484f
continuous wave Doppler
interrogation of tricuspid valve,
484f
descending aorta (DA) imaged at 0
and 90, 485f
left atrial appendage, 481f
left ventricular outflow tract view and
aortic valve, 483f
lower esophageal four-chamber view,
484f
mid-esophagus four-chamber view at
0, 481f
mitral valve view at 0 and 90, 483f
mitral valve view at 45 and 135, 483f
pulmonic valve and right ventricular
outflow tract, 484f
pulsed wave Doppler interrogation of
LAA, 482f
pulsed wave Doppler interrogation of
pulmonary vein, 482f
short-axis view of aortic valve, 483f
transgastric long-axis view of left
ventricle, 485f
transgastric short-axis view of left
ventricle, 485f
transgastric short-axis view of right
ventricle, 485f
Two-dimensional tomographic slices,
7980
Two-dimensional transthoracic
echocardiography
subcostal approach
abdominal aorta, 222f223f
Type B arch interruption, 1694
Type II tricuspid atresia, 1701
Type I tricuspid atresia, 1700
U
UCAs. See Ultrasound contrast agents
(UCAs)
Uhls anomaly, 1618, 1618f
Ultraharmonics, 421
Ultrasound
artifacts, 6163
basics of, 5564
definition of, 4
highly focused ultrasound, 1996
history of, 4
imaging by, 5760
A-mode, 57, 58f
B-mode, 57, 58f
M-mode, 58, 58f
resolution, 5860, 60f
transducers and probes, 58, 58f
2D ultrasound, 58, 58f
low-frequency ultrasound, 1996
microbubbles, 1996
nanodroplets, 1996
Index
problems, 1996
therapeutic applications, 1996
diagnostic ultrasound, 1996
tissue plasminogen activator, 1996
Ultrasound contrast agents (UCAs),
416417, 417f, 441. See also
Contrast echocardiography
commercially available, 417, 418t
idle, properties of, 417
safety of, 434435, 435t
ultrasound and, interaction between,
418419, 419f
use of, 428431
Ultrasound frequency, and Doppler
signal, 66
Ultrasound-guided compression therapy
of pseudoaneurysms, 699, 700f
Ultrasound-guided thrombin injection,
699
Ultrasound image, 87
Ultrasound stethoscope, 291. See also
Point-of-care diagnosis
Umbilical artery, pulse Doppler across,
1539f
Umbilical cord vessels, 1539f
Umbilical vein, pulse Doppler across,
1539f
Unicuspid aortic valve, 1619
Univentricular atrioventricular
connections, 16971700
Univentricular heart, in adults, 18451848
cardiac catheterization, 18471848
echocardiography, 18461847
transesophageal, 1847
MRI /CTA for, 1847
tricupsid atresia and post-fontan
adult, 1845, 1846f
Univentricular heart post-fontan
tricuspid atresia, 1848t
Unroofed coronary sinus, 1743
Unscrolled myocardial band model, 1182f
Untwisting (cardiac motion), 1183
during elongation, 1193
mitral valve opening and, 12001201
during postejection isovolumic phase
mirrors torsion, 1189
prominent left-sided vectors in,
11911193
torsion and, 11841185, 1200
Upper transesophageal and
transpharyngeal examination,
487506
arch vessels, identifications of, 488f
bilateral ostial vertebral artery
stenosis, 501f502f
carotid body paraganglioma,
detection of, 500f
Doppler signal of vessels on
transpharyngeal ultrasound, 488t
internal carotid artery stent,
detection of, 499f
left- and right-sided carotid arteries,
494f
left carotid artery stent, detection of,
498f
left carotid bulb and internal carotid
artery stenosis, 496f497f
left internal mammary artery, 493f
left-sided carotid arteries, 489f490f
left subclavian artery, 491f492f
left subclavian artery stenosis and
steal syndrome, 502f503f
left vertebral artery, 490f491f
left vertebral artery origin stenosis,
501f
pan-diastolic backflow in aortic arch
branches and neck vessels,
504f505f
right-sided carotid arteries, 495f, 496f
right subclavian artery, 494f
V
Valsalva aneurysm, sinus of, 16301632
Valsalva maneuver, 11261127
Valve perforation, 1045, 1046f
bicuspid aortic, 1046f
native mitral, 1046f, 1056f
Valvular aortic stenosis, 16181624
aortic root, 1621
aortic valve annulus, 1621
aortic valve area, 1622
associated anomalies in, 1624
critical neonatal aortic stenosis,
16221623
hemodynamics, 1623
severity of, 16211622
sinotubular junction, 1621
stenotic aortic valve, morphology,
16191620
transvalvular pressure gradients,
1622
vs. hypoplastic left ventricle, 1623
Valvular disease, in adults, 18131826
aortic valve, 18211826
mitral valve, 18181820
pulmonary valve, 18161818
tricuspid valve, 18131816
I-XLV
future directions
LV twist, evaluation of, 406
MRI tagging versus MRI velocity
vector imaging, 405406
three-dimensional STE, 404405, 405f
physics of strain and, 385, 389
and reproducibility and correlation
between vendors, 401404
Vena contracta area (VCA), 278
MR severity and, 869870
Vena contracta technique, 82
Vena contracta width, MR and, 868869
Ventricular arterial connection,
identification of, 15791582
concordant, 15801581
spatial relationship, 1582
Ventricular assist devices, 12221254
apical thrombi, 1230f
cardiac structure and function,
changes in, 12341240
clinical uses of, 12241226
dilated cardiomyopathy
baseline study, 1230f
with small secundum atrial septal
defect, 1232f
echocardiographic evaluation of,
12221254
explantation, 12491250
implantation by device strategy,
1226t
left ventricular over-filling, evidence
of, 12401246
levels of severity, 1225t
list of, 1229t
overview, 12221224
percutaneous continuous flow
devices, 12501252
Impella device, 1250, 1251f
TandemHeart, 12501251
postsurgical evaluation, immediate,
1234
preoperative echocardiographic
evaluation, 12291234
aorta, 1232
aortic valve, 1231, 1231f
atrial septum, 12311232
inferior vena cava, 1233
left atrium, 1231
left ventricle, 12291230
mitral valve, 1231
pericardium, 1231
pulmonic valve, 1232
right ventricle, 12331234
tricuspid valve, 1232, 1233f
I-XLVI
reverse remodeling, 1226
septal contour preleft, 1230f
Thoratec HeartMate II, 1223f
types of, 12261229
long-term axial flow devices, 1227
1228
long-term third generation
centrifugal flow systems, 12281229
short-term circulatory support,
12261227
ventricular size and function,
12341240
diastolic performance of ventricle,
12381240
inlet cannula, 1237, 1240f
left ventricle size and function,
12341237
motion of aortic valve, 1238
right ventricle size and function,
1237f, 1238
Ventricular deptal defect, 1581f
Ventricular morphology,
echocardiography of, 1579
Ventricular myxomas, 14681470
Ventricular papillary muscle rupture,
right
in female with dyspnea, 1014f1015f
transesophageal echocardiogram,
1013f1014f
Ventricular septal contour position, 1248
Ventricular septal defect (VSD), 1361,
1543f, 15911599, 17431746,
18021805
closure of, 557559, 560f
direction of shunt, 1597
Doppler evaluation of, 15961597
color Doppler, 15961597
pulsed and continuous wave Doppler
examination, 1597
3D TEE for, 513
midmuscular, 1544f
M-mode echocardiography, 1598
morphological location, 15911596
doubly committed subarterial
defects, 15941595, 1594f, 1596f
inlet ventricular septal defect,
15951596, 1595f
muscular ventricular septal defects,
15931594, 1593f, 1594f
perimembranous, 15921593, 1592f,
1595f, 1596f
objectives, 1591
pressure gradient across, 15971598
Qp/Qs ratios in, 1598
regurgitation and stenosis, 1598
restriction of, 1647f, 1648
stepwise evaluation for, 1591t
suitability for device closure, 1598
TGA and, 1658
transesophageal echocardiography
in, 1599
Ventricular septal defect closure, ICE
imaging during, 650, 651f
Ventricular septal defects, in adults,
18021805, 1806t
cardiac catheterization, 1805
closure of, 1805
echocardiography, 18041805
inlet, 1804
locations of, 1804f
membranous, 1803
MRI/CTA for, 1805
muscular, 1804
postoperative adult, 1805
supracristal, 1803
venturi effect, 1803f
Ventricular septal dropout, 1543f
Ventricular septal muscle rupture,
12951297
postmyocardial infarction, 1296f
transesophageal echocardiogram,
1013f1014f
Ventricular systolic function, assessment
of, traditional echocardiographic
limitations in, 381382, 381f
Ventricular tachycardia (VT), 1966
apical thrombi, 1966
coronary artery disease, 1966t
dilated cardiomyopathy, 1966t
echocardiography, 1966
hypertrophic cardiomyopathy, 1966t
left ventricle (LV), 1966
noncompaction, 1966t
sarcoid heart disease, 1966t
ventricular arrhythmias, 1966t
arrhythmogenic RV dysplasia
(ARVD), 1966
coronary artery disease, 1966
dilated cardiomyopathy, 1966
hypertrophic cardiomyopathy, 1966
infiltrative diseases, 1966
substrates, 1966
ventricular ectopy, 1966
common conditions, 1966t
Ventriculoarterial discordance, 1580f
Vertebral arteries, assessment of,
691693, 694f
Vijaya's echo criteria, 771, 771t
Visualization ,of received ultrasound
energy, 56, 56f
Volume rendering, of 3DE data set, 7879,
79f
V-ScanTM, 292, 292f
VSD. See Ventricular septal defect (VSD)
VVI. See Velocity vector imaging (VVI)
W
Wall motion abnormalities (WMA)
in DCM, 13711372
detection of, on echocardiogram, 226
in dilated cardiomyopathy, 1371
1372
ischemic, 12911292, 1292f1293f
squatting stress echocardiography in
left ventricular, 13241325
mechanism of, 1325
Wall motion score index (WMSI), 1313f,
1343
Wall motion scores (WMS), 1158
Wall visualization, 3DSE vs. 2DSE in, 1334
Watchman, 563
Wavelength, 55
Wide-angled display, 241
Wilkinss score, 536
William's syndrome, 1628f
WMA. See Wall motion abnormalities
(WMA)
WMS. See Wall motion scores (WMS)
WMSa. See WMS difference (WMSa)
WMS difference (WMSa), 1158
vs. summed difference score, 1159f,
1159t
WMSI. See Wall motion score index
(WMSI)
World Health OrganizationInternational
Society of Hypertension (WHO ISH), 294
Z
Zoom mode, 269