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Adult Congenital Heart Disease
Pankaj Madan, MD, MS, Medical Director
South Texas Adult Congenital Heart Center Methodist Hospital, San Antonio
Which statement is FALSE about congenital heart disease population?
1. More children than adults have congenital heart disease.
2. It is the fastest growing population of heart disorders in adults.
3. >90% of children born with congenital heart disease reach adulthood.
4. Adults with CHD require specialized care.
What is TRUE about Adult congenital heart disease care?
1. Most patients are seen in specialized ACHD clinics
2. Majority of patients are seen by pediatric cardiologists.
3. Majority of the patients are seeing adult cardiologists
4. Majority of the patients are lost to follow-up
What is true about ACHD subspecialty?
1. No specific training. Adult cardiologists are comfortable in taking care of ACHD patients.
2. No additional training required. Pediatric cardiologists are qualified to take care of ACHD patients.
3. Additional years of training beyond pediatric or adult cardiology required for expertise in ACHD care. There is additional board certification.
ACHD population
• 40,000 infants born with CHD/ year • THE most common birth defect • Successful outcome is a moving target
– Surviving initial surgical repair – Surviving to 1 year of age – Normal childhood – Normal adolescence
Survival to adulthood Survival through adulthood
Improving survival of CHD patients
0 20 40 60 80 100
1940
1960
1970
1980
1990
Percent survival to 18 years old
Decade born with CHD
ACHD: Population
Surviving to adulthood is now expected
Khairy et al. JACC 2010
Congenital Heart Disease population
70%
30%
PediatricAdult
1965
Congenital Heart Disease population
50%
50% PediatricAdult
2000
Congenital Heart Disease population
40%
60%
PediatricAdult
2010
Patients reaching Adulthood with CHD
325000
500000
750000
1000000
1400000
0
200000
400000
600000
800000
1000000
1200000
1400000
1600000
1970 1980 1990 2000 2010
40,000 new patients per year
More adults than children with CHD
Gilboa et al. Circulation 2016;134:101–109
Patients reaching Adulthood with CHD
325000
500000
750000
1000000
1400000
0
200000
400000
600000
800000
1000000
1200000
1400000
1600000
1970 1980 1990 2000 2010
40,000 new patients per year
55% have moderate to complex CHD
Gilboa et al. Circulation 2016;134:101–109
PROBLEM
0
1 –
–
0.9 –
–
0.8 –
–
0.7 –
SCD-Free Survival
(proportion)
Postoperative Interval (years) Silka et al. J Am Coll Cardiol . 1998; 32: 245-251.
5 10 15 20 25 30 35
TOF
d-TGA
COA
AS
n = 3589
Probability of SCD free survival after surgical correction
Age at death for adults with CHD
10
15
20
25
30
35
40
Tricuspid atresia TGA CoA
N=2609 patients 199 died Mean age at death for all diagnosis 37+/-15 years
Oehslin et al. Am J Cardiol 2000; 86: 1111
Mortality in Adult congenital heart disease
23%
24.50%
14.30%
7.10%
21.90%
9.20% Non cardiac
Heart Failure
Vascular
Perioperative
Arrhythmia
Others
Verheugt et al. EHJ 2010; 31: 1220-9
J Am Coll Cardiol 2007;50:1263–71)
Most of the mortality is cardiac related
Sudden cardiac death and heart failure leading causes of cardiac mortality
Morbidity in ACHD
050
100150200250300350400
Severe CHD Mild/moderateCHD
Normalpopulation
208
103
354
One year hospitalization rate of patients with severe and other cardiac lesions compared with the adult population of Quebec
(April 1999- March 2000)
Mackie AS. Am J Cardiol 2007; 99(6): 639-643
From: The Changing Demographics of Congenital Heart Disease Hospitalizations in the United States, 1998 Through 2010
JAMA. 2013;309(10):984-986. doi:10.1001/jama.2013.564
Patients reaching Adulthood with CHD
325000
500000
750000
1000000
1300000
0
200000
400000
600000
800000
1000000
1200000
1400000
1970 1980 1990 2000 2010
Once reaching adulthood • Survival is not as expected • Heart Failure and
arrhythmias • Morbidity is substantial
Long term complications
Adults with Congenital Heart Disease
Arrhythmias • Atrial • Ventricular • SCD
Heart Failure
Residual Shunts Valvular heart disease
Vascular lesions
Long term complications
Adults with Congenital Heart Disease
Arrhythmias • Atrial • Ventricular • SCD
Heart Failure
Residual Shunts Valvular heart disease
Vascular lesions
Adult Comorbidities • CAD, PVD • DM • OSA, COPD • Renal and Hepatic insufficiency
Diller et al. Circulation. 2015;132:2118-2125.
Sudden Cardiac death in ACHD patients
0
1
2
3
4
5
6
VSD CoA PS TOF D-TGA AS
Incidence / 1000 patient years
Silka et al. JACC 1998: 32: 245
Prevalence of Atrial arrhythmias in ACHD patients
0
10
20
30
40
50
60
TOF TGA Aorticstenosis
Pulmonarystenosis
ASD CoA Fontan
Vander Velde et al. Eur J Epidemiol 2005; 20: 549-557
40 5 10 15 20 25 30 35
Aortic coarction Tetralogy of Fallot
VSD Mustard-operation
Valvular disease Ebsteins anomaly Pulmonary atresia Fontan-operation
ASD (late closure) ccTGA
Complex anatomy Eisenmenger ANOVA P<0.0001
Mean ± SD 28.7 ± 10.4 25.5 ± 9.1 23.4 ± 8.9 23.3 ± 7.4 22.7 ± 7.6 20.8 ± 4.2 20.1 ± 6.5 19.8 ± 5.8 19.2 ± 6.2 18.6 ± 6.9 14.6 ± 4.7 11.5 ± 3.6
Peak VO2 (ml s/b mL)
Exercise intolerance in ACHD population
Diller GP, et al. Circulation 2005, 828-835.
MVO2 indicates Myocardial Oxygen Consumption; CCTGA, Congenitally Corrected Transposition of the Great Arteries; SD, Septal Defect; ANOVA, Analysis of the Variance; VO2, Volume of Oxygen
12
ACHD population • High risk group of young individuals
• Requires specialized and multidisciplinary care.
30
Who is providing the care? • Many being seen by Pediatric Cardiologists NOT
trained in ACHD
• Many being seen by Adult Cardiologists NOT trained in ACHD
• Few being seen in ACHD clinics
• Several have been lost to follow-up
31
ACHD Patients in USA vs Those in ACHD Clinics
Number Of Patients
800,000 – -
700,000 – -
600,000 – -
500,000 – -
400,000 – -
300,000 – -
200,000 – -
100,000 – -
0 –
800,000
Williams RG, et al. J Am Coll Cardiol. 2006;47(4):701-707. ACHA Clinic Directory Working Group 2007
60,000 in ACHD
Krasuski et al. Circulation. 2016;134:110–113
650 –
520 –
390 –
260 –
130 –
0 – < 6
Age Group
CHD
Pat
ient
s
6-12 13-17 18-22
Diagnosed by cardiologist
643 (100%)
n=643 (100%) n=643 (100%)
Seen by cardiologist 413 (64%)
Seen by cardiologist 292 (45%)
Seen by cardiologist 249 (39%)
n=466 (72%)
n=343 (53%)
Attrition 177 (28%)
53 (8%) Attrition 123 (19%)
51 (8%) Attrition 94 (15%)
The blue bars indicate patients who were not seen by a cardiologist within the indicated age range but were seen again by a cardiologist in an older age group (ie, transiently lost to follow-up).
Understanding Loss of CHD Follow-Up
Adapted from: Mackie A, et al. Circulation. 2009;120:302-309.
Reasons for being “Lost”
Patient Obstacles: • Patient assumes “cure” • Poor communication
from parents or pediatrician
• Loss of previous health records
• Gradual symptom onset Lack of health insurance
Physician Obstacles: • Physician assumes “cure” • Uninformed about
specific potential problems
• No prior records available • No reported symptoms • Symptoms ascribed to
more common causes
ACHD clinic: recurring themes
• Patients not aware of their medical and surgical history
• They receive inappropriate medical or surgical treatment
• Patients are misinformed.
Group 1: Simple CHD
• Unrepaired conditions: – Isolated small ASD – Isolated small VSD – Mild pulmonic stenosis
• Repaired conditions: PDA ASD VSD
Repaired or unrepaired conditions • Isolated aortic valve
disease • Isolated mitral valve
disease • Isolated patent foramen
ovale (PFO)
Patients should be seen at ACHD center at least once
32nd Bethesda Conference for care of ACHD
Group 2: Moderately complex CHD Repaired or Unrepaired: • Anomalous Left Coronary Artery from
Pulmonary Artery (ALCAPA)
• Anomalous pulmonary venous drainage (partial or total)
• Atrioventricular (AV) canal/septal defects (partial or complete)
• Ostium primum or sinus venosus ASDs.
• Coarctation of the aorta
• Ebstein’s anomaly
• Infundibular right ventricular outflow
obstruction (moderate to severe)
• Pulmonary valve regurgitation (moderate to severe)
• Pulmonic valve stenosis (moderate to severe)
• Sinus of Valsalva fistula/aneurysm
• Subvalvar or supravalvar aortic stenosis
• Tetralogy of Fallot
• VSD with any valve problems
Patients should be seen periodically at an ACHD center
32nd Bethesda Conference for care of ACHD
Group 3: Greatly complex CHD Repaired or Unrepaired: • Congenitally Corrected Transposition
of the Great Arteries (ccTGA or l-TGA) • Double outlet ventricle • Mitral atresia • Pulmonary atresia (all forms) • Pulmonary vascular obstructive
diseases • Shone’s Syndrome • Single Ventricle—all forms • Transposition of the Great Arteries
(d-TGA) • Tricuspid atresia • Truncus arteriosus/hemitruncus • Other abnormalities of AV
connections (i.e., crisscross heart, isomerism, heterotaxy syndromes)
All patients who have undergone the following procedures: • Arterial switch procedure • Blalock-Taussig shunt • Any Conduit(s), valved or nonvalved • Double-switch procedure • Fontan procedure • Mustard procedure • Norwood procedure • Rastelli procedure • Senning procedure
All Patients with Eisenmenger syndrome All Patients who are cyanotic (“blue”)
Patients should be seen regularly at ACHD center.
32nd Bethesda Conference for care of ACHD
When to refer
• If your patient had a surgery named after a physician.
• When more than one isolated structural defect is present.
• You can’t figure out anatomy in 5 seconds
• Your patient has never been seen by an ACHD physician
Additional material
Transplantation in ACHD
• Complex anatomy with multiple prior surgeries
• Highly sensitized
• Increased pulmonary vascular resistance
• Concomitant organ dysfunction e.g. liver
Transplant outcomes in CHD
Not all CHD are created equal
