REVIEW

CURRENT
OPINION Recent evolutions in pediatric and congenital
echocardiography
Heynric B. Grotenhuis and Luc L. Mertens

Purpose of review
Echocardiography is the first-line diagnostic technique in patients with congenital heart disease. Recent
developments include further standardization of pediatric and congenital echocardiography with general-
specific and lesion-specific guidelines. New research focuses on clinical validation of echocardiographic
quantitative techniques for assessing right ventricular and single ventricular function.
Recent findings
Recent guidelines include standardization of pediatric echocardiographic measurements and description of
utilization of imaging techniques in patients diagnosed with tetralogy of Fallot. Description of resource
utilization and organizational standards, including systems for quality assurance, are important tools for
improving the diagnostic quality of pediatric echocardiographic laboratories. We highlight interesting new
research on the echocardiographic assessment of right ventricular function in patients after tetralogy of
Fallot repair, patients with single-ventricle physiology and pediatric cardiomyopathies.
Summary
Pediatric and congenital echocardiography is evolving as an important specific area within
echocardiography. It is developing its own standards and quality control, and research in this field focuses
on development of more quantitative methods for assessing ventricular function.
Keywords
congenital heart disease, echocardiography, pediatric heart disease

INTRODUCTION echocardiographic study [2]. These guidelines serve

Two-dimensional and three-dimensional echo-
cardiography (2DE and 3DE) remain the first-line
diagnostic tool in the assessment of pediatric and
congenital heart disease (CHD). Its noninvasive
nature, accessibility and diagnostic accuracy are
the main reasons for this [1]. The field is maturing
quickly with establishment of professional guide-
lines, validation of measurements and the develop-
ment of normal values in the growing child. The last
few years research has focused on the application of
echocardiographic techniques in the quantitative
assessment of ventricular function and prognosis
in several CHD entities.
as a reference for pediatric echocardiography labora-
tories and their implementation enhances the level
of echocardiographic services provided [2,3]. The
use of standardized imaging techniques also facili-
tates exchange of information between laboratories
and multicenter research. The availability of ultra-
sound technology has resulted in a more widespread
use outside the more traditional indications, includ-
ing the use of focused echocardiography in neonatal
ICUs to guide hemodynamic management in pre-
term infants. The ASE, together with the European
Association for Echocardiography and the Associ-
ation for European Pediatric Cardiology, published

ORGANIZATION AND QUALITY
MONITORING OF A PEDIATRIC
ECHOCARDIOGRAPHY LABORATORY
The American Society of Echocardiography (ASE)
has published standards and recommendations on
how to perform a pediatric echocardiogram and
how to standardize measurements in a pediatric
Labatt Family Heart Center, Hospital for Sick Children, The University of
Toronto, Toronto, Ontario, Canada
Correspondence to Luc L. Mertens, Hospital for Sick Children, Labatt
Family Heart Center, 555 University Avenue, M5G 1X8, Toronto, ON,
Canada. Tel: +1 416 813 7418; fax: +1 416 813 5857; e-mail: luc.
mertens@sickkids.ca
Curr Opin Cardiol 2015, 30:118124
DOI:10.1097/HCO.0000000000000136

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January 2015

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 Latest in pediatric and congenital echocardiography Grotenhuis and Mertens
KEY POINTS

Echocardiography is the key imaging technique in
patients with pediatric and congenital heart disease.

Description of resource utilization and organizational
standards, including systems for quality assurance, are
important tools for improving the diagnostic quality of
pediatric echocardiographic laboratories.

Clinical validation of echocardiographic quantitative
techniques for assessing right ventricular and single
ventricular function has improved our understanding of
patients with congenital heart disease.
guidelines on the scope and clinical application of
this technique and the training requirements for
those performing the examinations [4]. Focused
echocardiography is expanding rapidly also in other
areas, including the pediatric emergency depart-
ment and the Pediatric Intensive Care Unit,
although no specific pediatric guidelines have been
developed yet.
Although other imaging techniques are devel-
oping, the role of echocardiography in the diagnosis
and management of specific CHD is becoming the
topic of more recent guidelines. The ASE published a
guideline on the role of multimodality imaging for
patients diagnosed with tetralogy of Fallot (TOF)
&&
[5 ]. Whereas echocardiography is most often suf-
ficient in infants with TOF, cardiac MRI (CMR) and
cardiac computer tomography may provide import-
&&
ant diagnostic information at later stages [5 ].
These guidelines provide a framework for rational
use of the different imaging modalities and it can be
expected that similar guidelines will be developed
for other CHD. Importantly, these guidelines also
include recommendations for adult CHD patients,
for whom no specific guidelines have been devel-
oped until now.
The ASE more recently also looked into the
organizational aspects of North American pediatric
&&
echocardiography laboratories. Lai et al. [6 ] pub-
lished the results of a survey among North American
pediatric echocardiography laboratory directors,
studying the organizational structure and clinical
productivity of academic pediatric echocardio-
graphy laboratories. The increasing need for
benchmarking is not only required by insurance
companies and accreditation bodies, but should also
allow more objective determination of staff require-
&&
ments in an echocardiography laboratory [6 ]. Pro-
ductivity measures per physician and sonographer
were reported, providing insight into workflow,
individual productivity and other duties like
research and on-call commitments per institution
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&&
[6 ]. The average number of studies per physician
was 15 per day, with a higher productivity in higher-
volume laboratories, and the average number of
studies performed per year by a sonographer was
&&
1297 studies [6 ]. The study was not intended to
generate recommendations for clinical staffing
requirements, but aimed to provide a framework
for future investigations into the optimal structure
and staffing of academic pediatric echocardio-
&&
graphy laboratories [6 ].
Quality improvement and prevention of diag-
nostic errors are also considered important aspects
&&
of our current clinical work environment [7 ].
&&
Building on previous work, Benavidez et al. [7 ]
reported risk factors associated with diagnostic
errors in the interpretation of echocardiography
studies in a large academic center. A very low error
rate of 0.17% was found, demonstrating the very
high diagnostic accuracy of pediatric echocardio-
graphy in a specialized tertiary care facility. Of all
detected errors, 77% were considered preventable
&&
[7 ]. Multivariate analysis identified patient weight
less than 5 kg to be a major risk factor, as well as rare
diagnosis, high anatomic complexity and studies
&&
performed after hours [7 ]. The study thereby
offered patient-related and situation-related factors
associated with diagnostic errors, which can serve as
potential targets for quality improvement. Continu-
ous quality improvement should be incorporated in
daily clinical practice and the authors provide
several suggestions to implement this, including a
systematic second reading of echocardiograms in
&&
neonates less than 5 kg [7 ]. Further study is needed
as this will require significant allocation of resources
and the costbenefit ratio will need to be deter-
mined.
PEDIATRIC NOMOGRAMS FOR CARDIAC
AND VASCULAR DIMENSIONS
In the context of continuous quality improvement
and standardization, availability of high-quality
normative data is important. One of the major
challenges in pediatric echocardiography is the
change in cardiac dimensions and functional
parameters in the growing child. How to normalize
measurements relative to changes in body size poses
a major challenge to the interpretation of echo-
cardiographic results, and z-scores have become
the generally accepted standard for expressing echo-
& &
cardiographic values [8 ,9 ]. The quality of the
z-scores is highly dependent on the quality of nor-
mal datasets and the generalizability of lab-specific
&
datasets to the more general population [9 ]. Mawad
&
et al. [9 ] published an in-depth review of statistical
methods used in studies describing pediatric
www.co-cardiology.com 119
 Pediatrics
echocardiographic reference values. When z-scores
are computed, adjustments should be made for any
differences in variability among subpopulations
(heteroscedasticity), as exemplified by the wider
range in aortic root dimensions for larger body
&
surface area (BSA) than for smaller BSA [9 ]. Critical
appraisal of any such differences in distribution or
other possible confounding factors is crucial for
&
correct presentation of normal values [9 ].
The ASE, in collaboration with the Pediatric
Heart Network, is currently developing universal
z-scores that should be applicable to laboratories
worldwide. In the meantime, only laboratory-
&
specific z-scores are available. Cantinotti et al. [8 ]
recently published nomograms in neonates and
infants, an age range which is of particular interest
given the effects of the fast somatic growth and the
fact that most critical CHD lesions present around
this time. Also, the clinical introduction of new
techniques like tissue Doppler and myocardial strain
imaging is often slowed due to the effort required
to establish normal reference values. Differences
in postprocessing algorithms between different
vendors further contribute to this [1013]. Levy
et al. [14] published a systematic review discussing
the normal ranges of right ventricular strain and
strain rate measurements in children. Normal mean
values were reported for right ventricular global
strain (29.0%; range 31.5 to 26.5%) and right
ventricular global systolic strain rate (1.9 s1; range
2.1 to 1.6 s1) [14]. Significant heterogeneity
and inconsistency were found between studies,
although meta-regression analysis showed that
these effects were not significant determinants of
variations among normal ranges of right ventricular
strain [14].
Other studies specifically focus on measure-
ments of right ventricular dimensions and function
in children. Right ventricular systolic function
can reliably be expressed by tricuspid annular peak
systolic velocity (S0 ) and tricuspid annular plane
systolic excursion (TAPSE), and pediatric reference
values have been published before [15,16].
Koestenberger et al. [17] recently published pediatric
z-scores of right ventricular outflow tract systolic
excursion in children (age 018 years), as an
additional marker of right ventricular function.
Right ventricular inflow and outflow function are
closely related in the normal heart, but this relation
is weak in TOF and arrhythmogenic right ventri-
cular dysplasia with frequent outflow tract patho-
logy [17]. Right ventricular outflow tract systolic
excursion therefore provides valuable insight in to
right ventricular performance and can be performed
in a simple and reproducible way using M-mode
[17].
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The complex right ventricular geometry limits
the utility of 2DE to reliably assess right ventricular
&
size and function. Renella et al. [18 ] studied the use
of 3DE in patients aged 020 years and reported that
acquisition of full-volume 3DE right ventricular
datasets could be successfully performed in the
majority of patients (96%) and datasets were meas-
urable from at least one view in 58% of cases. Right
ventricular volumetric assessment demonstrated
excellent reproducibility while being modest for
right ventricular ejection fraction, and older age
and female gender were risk factors for nonmeasu-
&
rable right ventricular datasets [18 ]. As echocardio-
graphic volumes differ from CMR volumes, with
lower volumes measured by echocardiography,
normal ranges need to be defined for 3DE.
NOVEL INSIGHTS IN TO VENTRICULAR
FUNCTION IN PATIENTS WITH PEDIATRIC
AND CONGENITAL HEART DISEASE
In the remaining sections we will focus on recent
research data looking into the use of echocardiog-
raphy techniques to assess ventricular function in
patients with pediatric and CHD. Three specific
topics are highlighted, although this only represents
a selection of recently published work and is
not exhaustive.
RIGHT VENTRICULAR ASSESSMENT IN
POSTOPERATIVE TETRALOGY OF FALLOT
TOF is the most common cyanotic CHD. Although
the lesion can be successfully surgically repaired
during the first year of life, a large number of
patients have residual lesions that put them at risk
for developing right ventricular and ultimately also
left ventricular dysfunction. Especially pulmonary
regurgitation and right ventricular outflow tract
stenosis are important lesions resulting in chronic
right ventricular volume and pressure loading,
respectively. Recently published ASE guidelines
about the appropriate use of imaging modalities
in TOF patients reserve a prominent role for echo-
&&
cardiography [5 ]. Due to the complex triangular
right ventricular shape, assessment of right ventric-
ular size and function remains difficult using 2DE
[19]. If 2D dimensions are used, measurements of
the right ventricular systolic and diastolic area from
the right ventricular centric apical four-chamber
view probably provide the best estimation of right
ventricular volumes [19]. Alghamdi et al. [20]
suggested that a right ventricular end-diastolic
volume more than 150 ml/m2 can reliably identify
patients with a right ventricular volume more
than 170 ml/m2 by CMR. 3DE can further aid in
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 Latest in pediatric and congenital echocardiography Grotenhuis and Mertens
determining biventricular size and function in TOF,
but one of its main limitations remains the feasi-
bility, especially in postoperative adults with poor
&&
acoustic windows [5 ,21].
Apart from volumetric assessment of right ven-
tricular ejection fraction, other techniques have
been studied in this population as well. Mercer-Rosa
&
et al. [22 ] looked at TAPSE as a parameter for global
right ventricular function in repaired TOF. TAPSE is
a quantitative measurement of right ventricular
longitudinal contraction, by measuring the distance
that the tricuspid valve moves towards the apex
&
using 2DE or M-mode [22 ]. As longitudinal right
ventricular shortening is an important component
of right ventricular systolic performance, TAPSE
appears to be a simple and reproducible quantitative
&
measurement [22 ]. When TAPSE was compared
with right ventricular ejection fraction measured
by CMR in a cohort of TOF patients, TAPSE did
not correlate with right ventricular ejection fraction
and correlated only weakly with right ventricular
&
stroke volume [22 ]. This contrasts with data pub-
lished by Koestenberger et al. [23], who concluded
that TAPSE correlated moderately with right
ventricular ejection fraction and right ventricular
end-diastolic volume. The relative weak correlation
data imply that the use of TAPSE as a surrogate
marker for right ventricular ejection fraction is pro-
bably limited, despite its high reproducibility
&
[22 ,23]. This can be explained by the fact that
TAPSE measures right ventricular function at the
right ventricular inlet, and TOF patients have fre-
quent regional functional abnormalities in the right
&
ventricular outflow tract [22 ,23]. The previously
discussed right ventricular outflow tract systolic
excursion may therefore be of additional value [17].
For studying regional right ventricular function,
strain and strain rate may provide additional infor-
&
mation [21]. Dragulescu et al. [24 ] described
regional abnormalities within the right ventricular
lateral wall with more significantly reduced right
ventricular apical longitudinal strain values com-
pared with longitudinal strain in the basal right
ventricular segments. Apart from the right ventric-
ular outflow tract, the right ventricular apex seems
to be affected more than the right ventricular base
&
[24 ]. Strain imaging was also used by the same
group to describe the effect of right bundle branch
block (RBBB) on regional mechanical activation
&&
[25 ]. A typical pattern of right ventricular electro-
mechanical dyssynchrony was found, manifested
electrically by RBBB and mechanically by early
septal activation and concomitant prestretch of
the lateral basal wall, followed by late contraction
and postsystolic shortening of the right ventricular
&&
lateral basal segment [25 ]. These abnormal
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segmental interactions result in an uneven distri-
bution of right ventricular wall stress and in reduced
&&
right ventricular contraction [25 ]. Postsystolic
right ventricular shortening does not contribute
to right ventricular ejection fraction as it occurs
after pulmonary valve closure, which also impedes
&&
diastolic filling [25 ]. A similar dyssynchrony pat-
tern was observed in patients with left bundle
branch block, with early activation of the septum
and late activation of the left ventricular lateral wall
and good response to cardiac resynchronization
&&
therapy [25 ]. Thambo et al. [26] suggested that a
similar response to biventricular pacing can be
observed in repaired TOF patients who develop
symptomatic right ventricular dysfunction.
Echocardiographic myocardial deformation
analysis has also demonstrated its use in the assess-
ment of unfavorable ventricular interactions
between the right ventricular and left ventricular.
Menting et al. [27] reported abnormal left ventricu-
lar rotational mechanics in TOF patients, which are
associated with right ventricular dilatation and
biventricular systolic dysfunction. The majority of
studied TOF patients (age 33  10 years) had lower
than normal left ventricular twist, mainly related to
decreased apical rotation [27]. These rotational
abnormalities also contribute to left ventricular
diastolic abnormalities in TOF and may be the actual
mechanistic link explaining the effect of right ven-
tricular dilatation on impaired left ventricular filling
&
[24 ]. 2D and 3D echocardiography are therefore
pivotal in the follow-up of biventricular function
and dimensions after repair of TOF.
ASSESSMENT OF SINGLE-VENTRICLE
HEMODYNAMICS AND PATHOLOGY
Advances in surgical techniques and perioperative
care over the past decade have greatly improved
survival of patients with single-ventricle physiology,
but morbidity and mortality remain significant
&&
[28 ]. Multiple risk factors for long-term single-
ventricle outcome have been identified, including
progressive ventricular dysfunction and tricuspid
&&
valve regurgitation [28 ]. 2DE is the main diagnos-
tic technique for single ventricle, but remains
largely qualitative given the absence of z-score
&&
values and the often complex anatomy [28 ].
Recent data looked into the feasibility of using
3DE. Marx et al. [29] analyzed 3D datasets for right
ventricular volumes and ejection fraction, obtained
in the context of a large multicenter study including
infants with hypoplastic left heart syndrome (HLHS)
who underwent two types of surgical palliation
[single ventricle reconstruction (SVR) trial compar-
ing the classical Norwood operation with the Sano
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 Pediatrics
modification]. The majority of datasets (75%) could
be analyzed and the results confirmed other
measurements of right ventricular function that
there were no significant differences in interstage
right ventricular volumes and ejection fraction [29].
Echocardiographic strain imaging has been used
to study global and regional function in single-
&&
ventricle patients. Tham et al. [28 ] used speckle
tracking to assess systolic ventricular function
throughout the different stages of palliation for
HLHS. Longitudinal and circumferential strain
and strain rate were measured, which progressively
&&
decreased after stage I [28 ]. Interestingly, right
ventricular adaptation to chronic systemic pressures
was characterized by a shift in the contraction pat-
tern from predominantly longitudinal to circum-
&&
ferential contraction of the single ventricle [28 ].
Li et al. [30] also used speckle tracking to study
differences in atrial and ventricular mechanics
between two types of Fontan procedures (atriopul-
monary versus extracardiac total cavopulmonary
connection). Significantly lower longitudinal, cir-
cumferential and radial systolic strain values were
observed in Fontan patients when compared with
healthy controls [30]. Fontan patients with an atrio-
pulmonary connection also had significantly lower
longitudinal and early diastolic strain rates than
patients with an extracardiac conduit, suggestive
of worse ventricular mechanics in the former group
[30].
Single-ventricle physiology depends on unob-
structed passive passage of blood from the systemic
circulation towards the lungs and single ventricle,
for which diastolic single-ventricle function is an
important component [31,32]. Increased ventricular
end-diastolic pressure is a marker for diastolic single-
ventricle dysfunction, and Husain et al. [31] studied
its correlation with strain and tissue Doppler in a
small group of single right ventricular patients
(median age 11.4 months). Simultaneously recorded
intracardiac pressures were correlated with early
diastolic strain rate, ratio of E wave inflow
velocity/early diastolic strain rate, and valve A
velocity, but not with tissue Doppler velocities
[31]. In contrast, Chowdhury et al. [32] found cor-
relations between tissue Doppler and invasive pres-
sure-volume loop analysis in a single-ventricle
population (age 3 months19 years), including tis-
sue Doppler E/A ratio of the septum, lateral ventric-
ular wall E/E ratio and tissue Doppler isovolumic
relaxation time (IVRT). Despite such discrepancy,
deformation assessment may be of additive value
to routine single-ventricle follow-up in identifying
subtle ventricular and hemodynamic dysfunction in
between stages of surgical single-ventricle pallia-
tion, and is therefore part of routine single-ventricle
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echocardiographic examination in our institution
&&
[28 ,31,32]. Serial echocardiography is an integral
part of the often intensive follow-up of single-ven-
tricle patients, and deformation imaging is recom-
mended to identify subtle functional abnormalities.
USE OF ECHOCARDIOGRAPHY IN
VARIOUS TYPES OF
CARDIOMYOPATHIES
Cardiomyopathy is a rare but serious condition in
childhood, which may be inherited or acquired
&&
[33 ,34]. Given the marked heterogeneity in
genetics, phenotype, rate of progression and prog-
nosis even among defined subtypes, continuous
follow-up after initial diagnosis is crucial, including
screening of family members for certain entities
&&
[33 ,34]. 2DE and M-mode are part of routine
and have been used as part of large multicenter
&&
outcome studies [33 ,35]. Lee et al. [35] recently
published a reproducibility study in children with
dilated cardiomyopathy (DCM), reporting good
reproducibility for left ventricular dimensions pro-
vided by both 2DE and M-mode. Interestingly, poor
correlation between M-mode and 2DE measure-
ments of shortening fraction were found, which
precludes the use of 2DE and M-mode interchange-
ably [35]. Using a consistent method for serial
measurements is therefore recommended, as is also
suggested by the most recent ASE guidelines, which
recommend the use of 2DE shortening fraction
[3,35].
The Pediatric Heart Network and the Pediatric
Cardiomyopathy Registry have performed ground-
breaking work in prospectively collecting clinical
and echocardiographic data for a better understand-
&& &&
ing of DCM [36 ,37]. Molina et al. [36 ] studied
predictors of disease progression, revealing older age
at diagnosis, larger left ventricular end-diastolic
dimension by M-mode and lower septal peak sys-
tolic tissue Doppler velocity as independent predic-
tors of disease progression and associated adverse
events. Increasing left ventricular end-diastolic
dimensions can be considered as part of the cardiac
remodeling process in patients with decreased left
&&
ventricular systolic function and DCM [36 ]. A
recent publication from the Pediatric Cardiomyop-
athy Registry reported mirror-image results, namely
younger age at presentation and lower left ventric-
ular end-diastolic dimension as predictors of recov-
ery within 2 years after DCM presentation [37].
Hypertrophic cardiomyopathy (HCM) has
also been studied as part of the Pediatric Cardio-
myopathy Registry, in which echocardiographic
left ventricular dimensions were an important
part to identify risk factors for death or heart
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 Latest in pediatric and congenital echocardiography Grotenhuis and Mertens
&&
transplantation [33 ]. Worse prognosis was found
in children presenting as infants, an underlying
metabolic disorder or mixed HCM with other car-
diomyopathy phenotypes like restricted or dilated
&&
cardiomyopathy [33 ]. Clinical evidence of conges-
tive heart failure and reduced left ventricular frac-
tional shortening by echocardiography predicted
&&
death or heart transplant [33 ]. The echocardio-
graphic phenotype of patients with pure HCM
was characterized by raised left ventricular fractional
shortening, increased left ventricular mass and septal
and posterior left ventricular wall thickness, as well as
diminished left ventricular end-diastolic dimension
&&
[33 ]. In contrast, HCM children with also features
of restrictive or dilated cardiomyopathy were charac-
terized by below-normal or normal left ventricular
fractional shortening and normal or raised left ven-
&&
tricular end-diastolic dimension [33 ]. Echocardiog-
raphy remains, therefore, crucial for determination
of prognosis in HCM, given the significance of diag-
nosis early in life and signs of any systolic left ven-
&&
tricular dysfunction [33 ].
Lastly, an important subgroup is children with
anthracycline-induced cardiomyopathy, being the
most important chronic morbidity among child-
hood cancer survivors [38]. Kocabas et al. [38] eval-
uated cancer patients (mean age 8.2 years) within
the first year of anthracycline treatment, reporting
predominantly right ventricular diastolic dysfunc-
tion even in patients who had received the lowest
cumulative dose. Tissue Doppler imaging showed
decreased E velocity and E/A ratio of tricuspid
valve and reduced S velocity of the mitral valve
in all three study groups with different instant
cumulative anthracycline doses [38]. Left ventri-
cular ejection fraction by 2DE was only mildly
decreased in the two higher-dose groups, when
the cumulative doses surpassed 120 mg/m2 [38]. Dias-
tolic function parameters by tissue Doppler appeared
to be more sensitive than standard echocardio-
graphic indices in detecting diastolic abnormalities
especially affecting the right ventricular. Commonly
used left ventricular ejection fraction and shorten-
ing fraction may fail to detect subtle alterations
in ventricular performance in this patient group,
and diastolic impairment even in the case of low
cumulative doses may precede chronic progressive
anthracycline cardiotoxicity at a later stage [38].
Despite the heterogeneity in disease types among
patients with cardiomyopathy, echocardiography
remains an important part of both clinical follow-
up and multicenter research initiatives.
CONCLUSION
Recent studies have provided a framework for
organization and the review process of a pediatric
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echocardiography laboratory, to ensure pro-
ductivity and quality. The use of more advanced
echocardiographic imaging techniques including
3DE, strain and tissue Doppler analysis has provided
important insight into different congenital and
acquired cardiac disease types with documentation
of even subtle cardiac abnormalities. Incorporation
of these techniques into clinical practice and future
multicenter research efforts should be considered
as a next step to further strengthen the role of
echocardiography as the key imaging technique in
patients with CHD.
Acknowledgements
None.
Financial support and sponsorship
None.
Conflicts of interest
There are no conflicts of interest.
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review and meta-analysis. J Am Soc Echocardiogr 2014; 27:549560.
15. Koestenberger M, Nagel B, Ravekes W, et al. Reference values of tricuspid
annular peak systolic velocity in healthy pediatric patients, calculation of z
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16. Koestenberger M, Ravekes W, Everett AD, et al. Right ventricular function in
infants, children and adolescents: reference values of the tricuspid annular
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score values. J Am Soc Echocardiogr 2009; 22:715719.
17. Koestenberger M, Ravekes W, Nagel B, et al. Reference values of the right
ventricular outflow tract systolic excursion in 711 healthy children and calcula-
tion of z-score values. Eur Heart J Cardiovasc Imaging 2014; 15:980986.
18. Renella P, Marx GR, Zhou J, et al. Feasibility and reproducibility of three-
& dimensional echocardiographic assessment of right ventricular size and
function in pediatric patients. J Am Soc Echocardiogr 2014; 27:903910.
A nice feasibility study of 3DE to assess right ventricular dimensions and function,
not previously performed in the pediatric population. 3DE is portrayed as a
reproducible imaging modality with a promising role for evaluation of congenital
heart disease.
19. Valsangiacomo Buechel ER, Mertens LL. Imaging the right heart: the use of
integrated multimodality imaging. Eur Heart J 2012; 33:949960.
20. Alghamdi MH, Grosse-Wortmann L, Ahmad N, et al. Can simple echocardio-
graphic measures reduce the number of cardiac magnetic resonance imaging
studies to diagnose right ventricular enlargement in congenital heart disease?
J Am Soc Echocardiogr 2012; 25:518523.
21. Villafane J, Feinstein JA, Jenkins KJ, et al. Adult Congenital and Pediatric
Cardiology Section, American College of Cardiology. Hot topics in tetralogy
of Fallot. J Am Coll Cardiol 2013; 62:21552166.
22. Mercer-Rosa L, Parnell A, Forfia PR, et al. Tricuspid annular plane systolic
& excursion in the assessment of right ventricular function in children and
adolescents after repair of tetralogy of Fallot. J Am Soc Echocardiogr 2013;
26:13221329.
This article reports on the clinical application and shortcomings of TAPSE in the
assessment of right ventricular function in TOF.
23. Koestenberger M, Nagel B, Avian A, et al. Systolic right ventricular function in
children and young adults with pulmonary artery hypertension secondary to
congenital heart disease and tetralogy of Fallot: tricuspid annular plane
systolic excursion (TAPSE) and magnetic resonance imaging data. Congenit
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124 www.co-cardiology.com
Copyright Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
24. Dragulescu A, Friedberg MK, Grosse-Wortmann L, et al. Effect of chronic
& right ventricular volume overload on ventricular interaction in patients
after tetralogy of Fallot repair. J Am Soc Echocardiogr 2014; 27:896
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An interesting study describing the distorted regional contraction pattern of the
right ventricle after TOF repair.
25. Hui W, Slorach C, Dragulescu A, et al. Mechanisms of right ventricular
&& electromechanical dyssynchrony and mechanical inefficiency in children after
repair of tetralogy of Fallot. Circ Cardiovasc Imaging 2014; 7:610618.
Excellent study looking at the mechanisms of right ventricular electromechanical
dyssynchrony after TOF repair.
26. Thambo JB, Dos Santos P, De Guillebon M, et al. Biventricular stimulation
improves right and left ventricular function after tetralogy of Fallot repair: acute
animal and clinical studies. Heart Rhythm 2010; 7:344350.
27. Menting ME, Eindhoven JA, van den Bosch AE, et al. Abnormal left ventricular
rotation and twist in adult patients with corrected tetralogy of Fallot. Eur Heart
J Cardiovasc Imaging 2014; 15:566574.
28. Tham EB, Smallhorn JF, Kaneko S, et al. Insights into the evolution of
&& myocardial dysfunction in the functionally single right ventricle between
staged palliations using speckletracking echocardiography. J Am Soc Echo-
cardiogr 2014; 27:314322.
Excellent study to describe the longitudinal systolic performance of single-ventricle
patients throughout all stages of surgical palliation by use of strain imaging,
providing insight in to the process of adaptation and remodeling of the single
ventricle to changes in loading conditions.
29. Marx GR, Shirali G, Levine JC, et al. Pediatric Heart Network Investigators.
Multicenter study comparing shunt type in the Norwood procedure for single-
ventricle lesions: three-dimensional echocardiographic analysis. Circ Cardio-
vasc Imaging 2013; 6:934942.
30. Li SJ, Wong SJ, Cheung YF. Atrial and ventricular mechanics in patients after
Fontan-type procedures: atriopulmonary connection versus extracardiac con-
duit. J Am Soc Echocardiogr 2014; 27:666674.
31. Husain N, Gokhale J, Nicholson L, et al. Noninvasive estimation of ventricular
filling pressures in patients with single right ventricles. J Am Soc Echocardiogr
2013; 26:13301336.
32. Chowdhury SM, Butts RJ, Buckley J, et al. MOCHA Investigators. Comparison
of pressure-volume loop and echocardiographic measures of diastolic func-
tion in patients with a single-ventricle physiology. Pediatr Cardiol 2014;
35:9981006.
33. Lipshultz SE, Orav EJ, Wilkinson JD, et al. Pediatric Cardiomyopathy Registry
&& Study Group. Risk stratification at diagnosis for children with hypertrophic
cardiomyopathy: an analysis of data from the Pediatric Cardiomyopathy
Registry. Lancet 2013; 382:18891897.
This is an excellent outcome study that defines prognostic factors for children with
hypertrophic cardiomyopathy, with a strong role for echocardiography.
34. Alexander PM, Daubeney PE, Nugent AW, et al. National Australian
Childhood Cardiomyopathy Study. Long-term outcomes of dilated cardio-
myopathy diagnosed during childhood: results from a national population-
based study of childhood cardiomyopathy. Circulation 2013; 128:2039
2046.
35. Lee CK, Margossian R, Sleeper LA, et al. Pediatric Heart Network Investi-
gators. Variability of M-mode versus two-dimensional echocardiography
measurements in children with dilated cardiomyopathy. Pediatr Cardiol
2014; 35:658667.
36. Molina KM, Shrader P, Colan SD, et al. Pediatric Heart Network Investigators.
&& Predictors of disease progression in pediatric dilated cardiomyopathy. Circ
Heart Fail 2013; 6:12141222.
Multicenter experience of predictors of disease progression in pediatric dilated
cardiomyopathy, identifying increased end-diastolic dimension and lower systolic
tissue Doppler indices by echocardiography to be predictors of disease progres-
sion and adverse events.
37. Everitt MD, Sleeper LA, Lu M, et al. Pediatric Cardiomyopathy Registry
Investigators. Recovery of echocardiographic function in children with idio-
pathic dilated cardiomyopathy: results from the pediatric cardiomyopathy
registry. J Am Coll Cardiol 2014; 63:14051413.
38. Kocabas A, Kardelen F, Ertug H, et al. Assessment of early-onset chronic
progressive anthracycline cardiotoxicity in children: different response
patterns of right and left ventricles. Pediatr Cardiol 2014; 35:8288.
Volume 30
Number 1
January 2015