Adult Congenital Heart Disease Pankaj Madan, MD, MS, Medical [PDF]

SLIDE 1

Adult Congenital Heart Disease

Pankaj Madan, MD, MS, Medical Director South Texas Adult Congenital Heart Center Methodist Hospital, San Antonio

SLIDE 2

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.

SLIDE 3

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

SLIDE 4

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
r adult cardiology required for expertise in

ACHD care. There is additional board certification.

SLIDE 5

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

SLIDE 6

Improving survival of CHD patients

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

SLIDE 7

ACHD: Population

Surviving to adulthood is now expected

SLIDE 8

Khairy et al. JACC 2010

SLIDE 9

Congenital Heart Disease population

70% 30%

Pediatric Adult

1965

SLIDE 10

Congenital Heart Disease population

50% 50%

Pediatric Adult

2000

SLIDE 11

Congenital Heart Disease population

40% 60%

Pediatric Adult

2010

SLIDE 12

Patients reaching Adulthood with CHD

325000 500000 750000 1000000 1400000

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

SLIDE 13

Patients reaching Adulthood with CHD

325000 500000 750000 1000000 1400000

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

SLIDE 14

PROBLEM

SLIDE 15

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

SLIDE 16

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

SLIDE 17

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

SLIDE 18

J Am Coll Cardiol 2007;50:1263–71)

Most of the mortality is cardiac related

SLIDE 19

Sudden cardiac death and heart failure leading causes of cardiac mortality

SLIDE 20

Morbidity in ACHD

50 100 150 200 250 300 350 400 Severe CHD Mild/moderate CHD Normal population

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

SLIDE 21

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

SLIDE 22

Patients reaching Adulthood with CHD

325000 500000 750000 1000000 1300000

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

SLIDE 23

Long term complications

Adults with Congenital Heart Disease

Arrhythmias

Atrial
Ventricular
SCD

Heart Failure

Residual Shunts Valvular heart disease Vascular lesions

SLIDE 24

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

SLIDE 25

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

SLIDE 26

Sudden Cardiac death in ACHD patients

1 2 3 4 5 6 VSD CoA PS TOF D-TGA AS

Incidence / 1000 patient years

Silka et al. JACC 1998: 32: 245

SLIDE 27

Prevalence of Atrial arrhythmias in ACHD patients

10 20 30 40 50 60 TOF TGA Aortic stenosis Pulmonary stenosis ASD CoA Fontan Vander Velde et al. Eur J Epidemiol 2005; 20: 549-557

SLIDE 28

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

f the Variance; VO2, Volume of Oxygen

12

SLIDE 29

ACHD population

High risk group of young individuals
Requires specialized and

multidisciplinary care.

SLIDE 30

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

SLIDE 31

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

SLIDE 32

650 – 520 – 390 – 260 – 130 – 0 –

< 6 Age Group CHD Patients 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.

SLIDE 33

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

SLIDE 34

ACHD clinic: recurring themes

Patients not aware of their medical and

surgical history

They receive inappropriate medical or

surgical treatment

Patients are misinformed.

SLIDE 35

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
vale (PFO)

Patients should be seen at ACHD center at least

nce

32nd Bethesda Conference for care of ACHD

SLIDE 36

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
bstruction (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

SLIDE 37

Group 3: Greatly complex CHD

Repaired or Unrepaired:

Congenitally Corrected Transposition
f 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

SLIDE 38

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

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SLIDE 42

Additional material

SLIDE 43

Transplantation in ACHD

Complex anatomy with multiple prior

surgeries

Highly sensitized
Increased pulmonary vascular resistance
Concomitant organ dysfunction e.g. liver

SLIDE 44

Transplant outcomes in CHD

SLIDE 45