Adult Congenital Heart Disease - OptumHealth Education · • Coarctation of the aorta •...

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