1. Adult Congenital Heart Disease Overview of the Population at Risk
Tampa St. Petersburg Land O’ Lakes
Adult Congenital Heart Disease Overview of the Population at Risk
Joel T. Hardin MD, FAAP, FACC, FAHA
Consultant Cardiologist, Adult Congenital Heart Disease

2. Metro Tampa Bay region ~2.9 million people.
~1% or 29,000 with congenital heart disease (CHD).
25% born with severe / potentially life-threatening defects, most requiring surgery during infancy.
75% of these have survived 50+ years later.
Approximately how many adults with history of severe CHD age 50+ live in our region?
100
15, ,000
150, ,000
Who knows, but I have 2 such patients in my practice

3. Metro Tampa Bay region ~2.9 million people.
~1% or 29,000 with congenital heart disease (CHD).
25% born with severe / potentially life-threatening defects, most requiring surgery during infancy.
75% of these have survived 50+ years later.
Approximately how many adults with history of severe CHD age 50+ live in our region?
100
15, ,000
150, ,000
Who knows, but I have 2 such patients in my practice

4. Congenital Heart Disease
Incidence: ~1% of live births (40,000 new patients/yr in the US).
40% of patients are diagnosed during infancy.
25% have potentially severe defects.
CHD accounts for 30-50% of birth defect related deaths.
90% of children with CHD survive into adulthood.
Longer-term survival rates are still significantly lower than the general population.
Increasing numbers of adult patients are presenting for initial and subsequent CHD interventions / surgery.

5. Long-Term CHD Outcomes
All CHD operations in Finland
13,876 operations were performed on 10,964 children < 15 yrs of age.
More males than females in all CHD diagnostic categories.
Excluding PDA ligation under age 1 month.
Data derived from a 98% follow-up rate among entire cohort.
Oldest patient 73 yr old.
367 patients older than 60 years.
Circulation. 2015;131:

6. Circulation. 2015;131:

7. Circulation. 2015;131:

8. Circulation. 2015;131:

9. Survival in different eras
Green: After 1989
Blue: – 1989
Y: Percent surviving
X: Years after surgery
Circulation. 2015;131:

10. ACHD: Numbers Rising
There are over 2,000,000 ACHD patients in the United States – this number is rising by ~5% per year.
ACHD population exceeds childhood CHD population in the United States.
Fewer than 30% of ACHD patients receive specialized care in ACHD clinical programs.
40% of patients with significant ACHD have experienced a gap in care of more than 3 yr.

11. Better but NOT Cured
Almost every childhood intervention for CHD renders improved health, but only a few interventions are truly curative.
Residual problems are common, and include ventricular dysfunction, valvular dysfunction, shunts, and arrhythmias.
These residua along with common CV risk factors contribute to increasing numbers of ACHD patients at risk for chronic heart failure.

12. High Complexity Conditions
Conduits, valved or nonvalved
Pulmonary atresia / intact septum
Cyanotic congenital heart disease
Tetralogy / pulmonary atresia
Double-outlet ventricle
Transposition of the great arteries
Eisenmenger syndrome
Tricuspid atresia
Fontan procedure
Other functional single ventricle
Truncus arteriosus
Other abnormalities of atrioventricular or ventriculoarterial connection
(i.e., crisscross heart, heterotaxy syndromes, ventricular inversion)

13. High Complexity Conditions
Frequent follow-up (generally every 6 – 12 months) at a regional ACHD center.
Stabilized ACHD patients with complex disease who require hospitalization for urgent or acute care should generally be transferred to a regional ACHD center.

14. Moderate Complexity Conditions
Aorto–left ventricular fistulas
Pulmonary valve regurgitation (moderate to severe)
Anomalous pulmonary venous connection, partial or total
Pulmonary valve stenosis (moderate to severe)
Atrioventricular septal defects (partial or complete)
Sinus of Valsalva fistula/aneurysm
Coarctation of the aorta
Sinus venosus atrial septal defect
Ebstein’s anomaly
Subvalvar AS or SupraAS (except HOCM)
Infundibular right ventricular outflow obstruction of significance
Tetralogy of Fallot
Ostium primum atrial septal defect
VSD with: absent valve or valves, aortic regurgitation, coarctation of the aorta, mitral disease, right ventricular outflow tract obstruction, stradding tricuspid/mitral valve
PDA (not closed)

15. Moderate Complexity Conditions
Regular follow-up (generally every months) at a regional ACHD center.
Stabilized ACHD patients with moderate disease who require hospitalization for urgent or acute care should be transferred to a regional ACHD center or should receive remote consultation from an ACHD specialist.

16. Low Complexity Conditions
Native disease
Repaired conditions
Isolated congenital aortic valve disease
Previously ligated or occluded PDA
Isolated congenital mitral valve disease (e.g., except parachute valve, cleft leaflet)
Repaired ASD without residua
Isolated PFO or small ASD
Repaired VSD without residua
Isolated small VSD
Mild pulmonary stenosis
Small PDA
ACHD center consultation at least once to formulate future needs for follow-up. An ACHD specialist should be notified or consulted when a patient with low complexity CHD is admitted to a non-ACHD center.

17. ACHD Hospitalization Trends
J Am Coll Cardiol 2009;54:460–7)

18. Surgery in Adults with CHD
Spans all ages and often performed in the context of multiple co-morbidities.
Common ACHD surgical admissions:
Tetralogy of Fallot
Aortic stenosis
Atrial septal defects
Coarctation of the aorta
Pulmonic valve stenosis
Ebstein’s anomaly of tricuspid valve
Single ventricles
Transposition of great arteries s/p atrial baffle procedures

19. Prevalence of ACHD Interventions
At least 25% of ACHD patients require a surgical or catheter-directed intervention.
40% are re-interventions after childhood
70% surgical cases % catheter-directed
Around 17% of ACHD patients require more than one surgery or transcatheter intervention.
20% of surgical ACHD admissions are patients with tetralogy of Fallot (37% for pulmonic valve replacement).
Circulation. 2011;124:

20. Mortality Risk in ACHD Surgery
Survival after INITIAL surgery for CHD as an adult is comparable to patients who were never operated on.
Common “late” interventions: ASD, isolated aortic valve stenosis, Marfan syndrome
After 3rd or more surgeries, mortality was 2-3 times higher than patients never operated on.
Men with reoperation for CHD in adulthood had a 2-times-higher risk of death compared with women.
Circulation. 2011;124:

21. Multidisciplinary ACHD Team
Primary care / cardiovascular internist
Consultant ACHD cardiologist and APN
Imaging specialist with CHD expertise
Interventional cardiologist with CHD expertise
Electrophysiologist with CHD expertise
Congenital heart surgeon
Cardiovascular anesthesiologist with CHD expertise

22. Additional Stakeholders
Advanced heart failure / transplant team
Pulmonary arterial hypertension specialist
Pulmonary medicine / critical care
Maternal-fetal medicine
Hepatology
Nephrology
Genetics consultant

23. Where to Care for ACHD Patients
Adult hospital with collaboration from congenital heart disease specialists and congenital heart surgeons.
Pediatric hospital with collaboration from consultants in internal medicine specialties.

24. ACHD Board Certification
ABIM sponsored exam leading to ACHD certification.
Currently 195 diplomates after first exam in 2015
Board-certified cardiologists (ABIM) and pediatric cardiologists (ABP) can apply for ACHD certification.
“Practice Pathway” option until 2019 for those already holding ABIM or ABP certification prior to 2016: For full-time physicians, at least 3 of 5 past years (including year prior to application), 40% of post-training clinical practice time or 25% of their total post-training professional time in ACHD clinical practice.
“ACHD fellowship training pathway”

26. Tampa St. Petersburg Land O’ Lakes
2 board-certified / 1 board-eligible ACHD cardiologists, 1 ACHD physician assistant.
3 congenital heart surgeons.
2 cardiac anesthesiologists specializing in congenital heart disease.
2 interventional cardiologists specializing in congenital heart disease.
Cardiac electrophysiology consultants.
Advanced cardiac imaging consultants.
Advanced heart failure consultants.
Adult Congenital Heart Association Comprehensive Care Center accreditation in progress.
Office Ph: (813) or (727)
Joel T. Hardin MD: Mobile (312) , FAX (888)