Evolution of pediatric cardiac surgery

and the cardiovascular health imperative

Charles D. Fraser, Jr., MD

Director, Texas Center for Pediatric and Congenital Heart Disease
Professor of Surgery and Pediatrics
University of Texas Dell Medical School
Dell Children’s Medical Center - Austin, Texas
 ~1 in 100 newborns
will have CHD

25%

1/4 will need intervention

in the first year of life
Texas Center for Pediatric and Congenital Heart Disease
Texas Center for Pediatric and Congenital
Heart Disease Team
Treatment of transposition of the great
arteries – a paradigm of success
The amazing story of surgery for cyanotic
children (“blue babies”) – Johns Hopkins
Developing cardiopulmonary Bypass

On May 6, 1953, John H. Gibbon Jr., MD performed the first successful

human heart surgery assisted by a heart-lung machine.
Intra-cardiac congenital heart surgery

C. Walton Lillehei, MD John W. Kirklin, MD

Early cardiopulmonary Bypass

DeWall-Lillehei pump oxygenator 1955

D-TGA
with IVS

© 2015 Texas Children’s

Hospital
Transposition Outcomes
TGA ‘85-’89 “ASO”

TGA/IVS ’74-’84
Recent “Mustard Era”

TGA/IVS ‘63-’73
“Mustard Era”

TGA/IVS TGA
‘57-’64 ‘57-’64

Heart Disease in Infants, Children, and Adolescents Including the

Fetus and Young Adult, 8th Edition, Vol II, Edited by Hugh Allen,
Early Efforts
Blalock – Hanlon Procedure

Doty – Cardiac Surgery Operative Technique

Blalock – Hanlon Procedure

Doty – Cardiac Surgery Operative Technique

Senning Operation
Arterial Switch Procedure –
anatomic/physiologic
correction
 Anatomic
Correction of
Transposition of
the Great Arteries
Adib D. Jatene, M.D. (by invitation),
V.F. Fontes, M.D. (by invitation),
L.C.B. Souza, M.D. (by invitation),
P.P. Paulista, M.D. (by invitation),
C. Abdulmassih Neto, M.D. (by invitation),
and J.E.M.R. Souza, M.D. (by invitation), São
Paulo, Brazil,
Sponsored by E.J. Zerbini, M.D.,
São Paulo, Brazil

J Thorac Cardiovasc Surg 83:20-26, 1982 Adib Domingos Jatene

J Thorac Cardiovasc Surg 83:20-26, 1982
© 2015 Texas Children’s Hospital
© 2015 Texas Children’s Hospital
© 2015 Texas Children’s Hospital
© 2015 Texas Children’s Hospital
© 2015 Texas Children’s Hospital
© 2015 Texas Children’s Hospital
© 2015 Texas Children’s Hospital
760g Tiny Baby
Preterm 760 grams
• 5 day old girl
• Smallest of
spontaneous
triplets
• C-section at 28w, 1d
• Desaturated to 60s,
not responding
to supplemental oxygen
• TGA-IVS & PFO diagnosed on ECHO
Infant / Neonatal Arterial Switch Operation
at Texas Children’s Hospital by Year (1995-2018)
35
N = 394 29 29
30
25
20 21
1918 1918
20 1718 17 17 17
15 1516
15 1314 12 12 13
10 10
10
5
5
0
6/1/95 –
03/28/18
 Infant / Neonatal ASO at TCH June 1995 – Nov 2017
Overall Survival

Survival
5y – 98% Median f/u
10y – 98% 8y (7d – 21y)
15y – 98%
N=377

6/1/95 – 11/28/17
Why Have We Done
So Well?

Tenacious Pursuit
of Excellence –
International relationships
1984 - 1985
 “Outcomes in congenital heart surgery are optimized by
surrounding the patient with the greatest expertise possible
during all times of the surgical and postoperative period.”

RBB Mee
December, 1991
The Rainbow Fish - By Marcus Pfister
The Playbook
CVOR Team
Peritoneal Dialysis Catheter (PD Cath)
Baseline Values
Safe, Consistent Pre/Post-op Care
“If you want to go fast, go alone . . .
If you want to go far go together.”

African Proverb
What’s Next ?
Data and Statistics on Congenital Heart Defects
 What happens to adults after the ASO ?
Kempny A, Wustmann K, Borgia F, Dimopoulos K, Uebing A,
Li W, Chen SS, Piorkowski A, Radley-Smith R, Yacoub MH,
Gatzoulis MA, Shore DF, Swan L, Diller GP
Outcome in Adult Patients After Arterial Switch Operation for Transposition of the Great Arteries.

• 145 adult patients (age>16y) with ASO followed

• 3 died, 3 coronary lesions, 56% aortic root
dilation, 42% residual lesions/required
reintervention
• Periodic noninvasive surveillance is safe
Kempny A et al. Int J Cardiol. 2013 Sep 10;167(6):2588-93.
The need for radical solutions
 HLHS

Barron et al, Lancet 2009; 374: 551–64

 Classic
Norwood
(w/ BT shunt)

rron et al, Lancet 2009; 374: 551–64

 Mod.
Norwood
(RV-PA
conduit)

Barron et al, Lancet 2009; 374: 551–64

 Glenn

Barron et al, Lancet 2009; 374: 551–64

 Extracardiac
Fontan

Barron et al, Lancet 2009; 374: 551–64

Transplant-Free Survival and Interventions at 6 Years in the SVR Trial.
Circulation. 2018 May 22;137(21):2246-2253. doi: 10.1161/CIRCULATIONAHA.117.029375.
Epub 2018 Feb 1.

(in children with HLHS undergoing staged palliation)…

transplant-free survival for the RVPAS versus modified
Blalock-Taussig shunt groups did not differ at 6 years (64%
versus 59%, P=0.25) or with all available follow-up of
7.1±1.6 years (log-rank P=0.13).
First Clinical LVAD Success
• DeBakey, 1966
• 37-year-old woman
• Post-cardiotomy
• Supported for 10
Days
• Survived long-term
post-transplant
• Died after motor-
vehicle accident

Content courtesy of OH “Bud” Frazier, MD

 Artificial Heart Development
• 1963 - National Advisory
Heart Council gives high
priority to artificial heart
development
• 1965 – Texas Heart
Institute received
$500,000 from Congress
for artificial heart
development

36th POTUS, near-fatal heart attack in 1955

Content courtesy of OH “Bud” Frazier, MD

 1969
THI
Cooley/Liotta first TAH
 Texas Children’s
Hospital VAD Experience – CDF lessons learned:
1995-2018

Content courtesy of Texas Children’s Hospital

Heart Failure in Children – historical options
 DeBakey Child
2004-2005

• N=3
• Median age, 13 years (6-16)
• Support duration:
– Median 18 days (16 – 84)
• Outcomes
– 2 bridge-to-transplant
– 1 died
• First attempt of an exclusively
pediatric VAD

Morales DL et al.,. J Heart Lung Transplant. 2005 Mar;24(3):331-7

Content courtesy of Texas Children’s Hospital
© 2009 Texas Children’s Hospital
Content courtesy of Berlin Heart
Applying adult technology to help children
First Pediatriac VAD Placement at DCMC
09/24/19
Advances in Bioengineering
First VAD Placement at DCMC 09/24/19

A. Pre-Heartware II (LVAD) B. Post-Heartware II (LVAD)

Implant. 9.23.2019 Implant. 9.24.2019
AHA STATISTICAL UPDATE

Heart Disease and Stroke Statistics— 2019 Update

A Report From the American Heart Association
Chart 13-3. Deaths attributable to cardiovascular disease (United States, 1910–2016).
Cardiovascular disease (International Classification of Diseases, 10th Revision codes I00–I99) does not include congenital heart disease. Before 1933, data are for a
death registration area and not the entire United States.
Source: National Heart, Lung, and Blood Institute.
Heart Disease Strikes Back Across the
U.S.; Even in Healthy Places

Wall Street Journal

January 15, 2020
Death from Cardiovascular Disease Rising
• Death rates from cardiovascular
disease among people between the
ages of 45 and 64 are rising all across
America.
• The South and some other parts of the
nation have perpetually high rates of
death from heart disease and strokes,
middle-aged cardiovascular death
rates are rising even in places where
those rates have been historically low
Teaching Early
• More than a quarter of elementary-school children
screened in the 2018-2019 school year were overweight or
obese, and 19.2% had borderline high cholesterol,
according to program data from Healthy Hearts
• Healthy Hearts – a cardiovascular-disease screening and
education program for school-age children and families
• Teach healthy lifestyle and detect factors early
AHA STATISTICAL UPDATE

Heart Disease and Stroke Statistics— 2019 Update

A Report From the American Heart Association
Chart 13-4. Percentage breakdown of deaths attributable to cardiovascular disease (United States, 2016).
Total may not add to 100 because of rounding. Coronary heart disease includes International Classification of Diseases, 10th Revision (ICD-10) codes I20 to I25;
stroke, I60 to I69; heart failure, I50; high blood pressure, I10 to I15; diseases of the arteries, I70 to I78; and other, all remaining ICD-I0 I categories.
Source: National Heart, Lung, and Blood Institute from National Center for Health Statistics reports and data sets.
“There can be no keener revelation of a
society's soul than the way in which it
treats its children.”

Nelson Mandela
Benjamin et al. Circulation 2018;137:e67-e492.
Patient with transposition of the great arteries
Modified from: www.determinantsofhealth.org
Texas Center for Pediatric & Congenital
Heart Disease

To redesign the care of patients and families with

CHD by developing a model system of health
delivery that is integrated, holistic, patient-
centered, value-oriented, and technology-driven.
 Health Transformation and Design Program
at the Texas Center for Pediatric and Congenital Heart Disease

Outcomes measurement, research, and quality

improvement initiatives to improve the care of patients
and families with CHD while focusing on projects that
have a high impact in a relatively short timeframe.
Clinical Research and Outcomes
Clinical Research and Outcomes
• Identification of meaningful outcomes
– Roadmap initiative (Design and Value Institutes)

• Measurement of outcomes
– Registries / short-term outcomes
– Long-term follow-up (survival, QOL, neurodevelopment,
psychosocial issues) – collaboration with Value Institute

• Improvement of outcomes