Adult Congenital Heart Disease Pankaj Madan, MD, MS, Medical - PowerPoint PPT Presentation

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 1990 1980 Decade born 1970 with CHD 1960 1940 0 20 40 60 80 100 Percent survival to 18 years old

ACHD: Population Surviving to adulthood is now expected

Khairy et al. JACC 2010

Congenital Heart Disease population 30% Pediatric 70% Adult 1965

Congenital Heart Disease population 50% Pediatric 50% Adult 2000

Congenital Heart Disease population 40% Pediatric Adult 60% 2010

Patients reaching Adulthood with CHD 1600000 More adults than 1400000 1400000 children with CHD 1200000 1000000 1000000 800000 750000 600000 40,000 new 500000 patients per year 400000 325000 200000 0 1970 1980 1990 2000 2010 Gilboa et al. Circulation 2016; 134 :101–109

Patients reaching Adulthood with CHD 1600000 1400000 1400000 55% have moderate to 1200000 complex CHD 1000000 1000000 800000 750000 600000 40,000 new 500000 patients per year 400000 325000 200000 0 1970 1980 1990 2000 2010 Gilboa et al. Circulation 2016; 134 :101–109

PROBLEM

Probability of SCD free survival after surgical correction 1 – – TOF COA 0.9 – SCD-Free Survival – (proportion) d-TGA 0.8 – AS n = 3589 – 0.7 – 0 10 15 20 25 30 35 5 Postoperative Interval (years) Silka et al. J Am Coll Cardiol . 1998; 32: 245-251.

Age at death for adults with CHD 40 35 N=2609 patients 30 199 died 25 Mean age at death for all diagnosis 37+/-15 20 years 15 10 Tricuspid atresia TGA CoA Oehslin et al. Am J Cardiol 2000; 86: 1111

Mortality in Adult congenital heart disease 9.20% 23% Non cardiac 21.90% Heart Failure Vascular 7.10% 24.50% Perioperative 14.30% Arrhythmia Others Verheugt et al. EHJ 2010; 31: 1220-9

Most of the mortality is cardiac related J Am Coll Cardiol 2007;50:1263–71)

Sudden cardiac death and heart failure leading causes of cardiac mortality

Morbidity in ACHD One year hospitalization rate of patients with severe and other cardiac lesions compared with the adult population of Quebec (April 1999- March 2000) 354 400 350 208 300 250 200 103 150 100 50 0 Severe CHD Mild/moderate Normal CHD population 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 1400000 Once reaching adulthood 1300000 1200000 1000000 1000000 800000 • Survival is not as expected 750000 600000 • Heart Failure and 500000 400000 325000 arrhythmias 200000 • Morbidity is substantial 0 1970 1980 1990 2000 2010

Long term complications Arrhythmias Heart Failure • Atrial • Ventricular • SCD Adults with Congenital Heart Disease Vascular lesions Residual Shunts Valvular heart disease

Long term complications Arrhythmias Heart Failure Adult Comorbidities • Atrial • Ventricular • CAD, PVD • SCD Adults with • DM Congenital • OSA, COPD Heart Disease • Renal and Hepatic insufficiency Vascular lesions Residual Shunts Valvular heart disease

Diller et al. Circulation . 2015;132:2118-2125.

Sudden Cardiac death in ACHD patients 6 5 Incidence / 4 1000 patient years 3 2 1 0 VSD CoA PS TOF D-TGA AS Silka et al. JACC 1998: 32: 245

Prevalence of Atrial arrhythmias in ACHD patients 60 50 40 30 20 10 0 TOF TGA Aortic Pulmonary ASD CoA Fontan stenosis stenosis Vander Velde et al. Eur J Epidemiol 2005; 20: 549-557

Exercise intolerance in ACHD population Mean ± SD Aortic coarction 28.7 ± 10.4 Tetralogy of Fallot 25.5 ± 9.1 VSD 23.4 ± 8.9 Mustard-operation 23.3 ± 7.4 Valvular disease 22.7 ± 7.6 Ebsteins anomaly 20.8 ± 4.2 20.1 ± 6.5 Pulmonary atresia 19.8 ± 5.8 Fontan-operation 19.2 ± 6.2 ASD (late closure) 18.6 ± 6.9 ccTGA 14.6 ± 4.7 Complex anatomy 11.5 ± 3.6 Eisenmenger ANOVA P <0.0001 5 10 15 20 25 30 35 40 Peak VO 2 (ml s/b mL) MVO 2 indicates Myocardial Oxygen Consumption; CCTGA, Congenitally Corrected Transposition of the Great Arteries; SD, Septal Defect; ANOVA, Analysis of the Variance; VO 2 , Volume of Oxygen 12 Diller GP, et al . Circulation 2005, 828-835 .

ACHD population • High risk group of young individuals • Requires specialized and multidisciplinary care.

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 30

ACHD Patients in USA vs Those in ACHD Clinics 800,000 800,000 – - 700,000 – - 600,000 – - 500,000 – Number - Of Patients 400,000 – - 300,000 – - 200,000 – - 100,000 – 60,000 in ACHD - 0 – Williams RG, et al. J Am Coll Cardiol . 2006;47(4):701-707. Krasuski et al. Circulation. 2016;134:110–113 31 ACHA Clinic Directory Working Group 2007

Understanding Loss of CHD Follow -Up n=643 (100%) n=643 (100%) 650 – Attrition 177 (28%) 520 – n=466 (72%) CHD Patients 53 (8%) Attrition 390 – 123 (19%) n=343 (53%) Diagnosed Attrition 51 (8%) by cardiologist 94 (15%) 643 (100%) 260 – Seen by Seen by cardiologist cardiologist Seen by 413 (64%) 292 (45%) 130 – cardiologist 249 (39%) 0 – < 6 6-12 13-17 18-22 Age Group 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). Adapted from: Mackie A, et al. Circulation. 2009;120:302-309.

Reasons for being “Lost” Patient Obstacles: Physician Obstacles: • Patient assumes “cure” • Physician assumes “cure” • Poor communication • Uninformed about from parents or specific potential pediatrician problems • Loss of previous health • No prior records available records • No reported symptoms • Gradual symptom onset • Symptoms ascribed to Lack of health insurance 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: Repaired or unrepaired conditions – Isolated small ASD – Isolated small VSD • Isolated aortic valve Patients should be seen – Mild pulmonic stenosis disease • Isolated mitral valve at ACHD center at least • Repaired conditions: disease once PDA • Isolated patent foramen ovale (PFO) ASD VSD 32 nd Bethesda Conference for care of ACHD

Group 2: Moderately complex CHD Repaired or Unrepaired: • Pulmonary valve regurgitation • Anomalous Left Coronary Artery from (moderate to severe) Pulmonary Artery (ALCAPA) • Anomalous pulmonary venous drainage • Pulmonic valve stenosis (moderate to (partial or total) Patients should be seen severe) • Atrioventricular (AV) canal/septal defects (partial or complete) • Sinus of Valsalva fistula/aneurysm periodically at an ACHD • Ostium primum or sinus venosus ASDs. • Subvalvar or supravalvar aortic • Coarctation of the aorta center stenosis • Ebstein’s anomaly • Tetralogy of Fallot • Infundibular right ventricular outflow obstruction (moderate to severe) • VSD with any valve problems 32 nd Bethesda Conference for care of ACHD