1. Adult Congenital Heart: Current Status & Long Term Issues
25th Annual CV Symposium
Marc D. Knepp, MD
November 4, 2016

2. I HAVE No FINANCIAL DISCLOSURES

3. Objectives
Define the current status of Congenital Heart Disease in the Adult
Identify co-morbidities of Grown Up Congenital Heart Disease
Discuss the importance of long term follow up and disease specific counseling

5. Congenital Heart Disease
Malformation of the heart present at birth
Most common birth defect
8 per 1000 live births
Common Forms
Ventricular Septal Defect (VSD)
Atrial Septal Defect (ASD)
Tetralogy of Fallot (TOF)
Transposition of the Great Arteries (TGA)
10% diagnosed as an Adult

6. Treatment of CHD Medical therapy only temporizes
50% survival to adulthood
First surgical intervention, 1938
PDA ligation, Gross & Hubbard, Children’s Boston
Universally fatal until the 1940s
Shunt surgery devised by Dr. Helen Taussig
Performed by Dr. Arthur Blalock
November 1944, Johns Hopkins

7. Early Congenital Heart Surgery
1954– Dr. C.W. Lillehei performed the first TOF repair
Cross Circulation

8. Cross Circulation

9. Cardio-Pulmonary Bypass
Revolutionized the Care of CHD

10. Survival of Unrepaired Tetralogy of Fallot
Betranou EG, Blackstone EH, Hazelrig JB, Kirklin JW. Life expectancy without surgery in tetralogy of Fallot. American Journal of Cardiology 1978

11. Survival of Repaired TOF
Murphy JG, Gersh BJ, Mair DD et al. Long-term outcome in patients undergoing surgical repair of tetralogy of Fallot. N Engl J Med 1993: 329:

12. But some work yet to do…..
Nollert et al. Long-Term Survival in Patients With Repair of Tetralogy of Fallot: 36-Year Follow-Up of 490 Survivors of the First Year After Surgical Repair. JACC 1997:

13. Survival doesn’t mean cured
In 2015, >90% of newborns with CHD will live until adulthood
Even with successful neonatal or childhood repairs, many will still need procedures and follow up thru life

14. Diller et al Circulation 2015

15. Diller et al Circulation 2015

16. Cause of Death

19. Adult Congenital in the US
Over 1.3 million Adult Congenital patients
>50% of all congenital heart patients
35-40,000 CHD pts/yr turn 18
18 different distinct Congenital Heart Lesions
Adults with CHD have 3-4x higher rates of ER visits, hospitalizations, and ICU admits, compared to general population 1.
2. Facing Lifesaving Heart Surgery, Twice. Wall Street Journal, 8/20/12

20. Annual ACHD Hospitalizations
Agarwal et al. JAHA 2016

21. Age of Admitted ACHD Patient

22. Cost of Hospital ACHD Care

23. Long Term Follow Up Issues
Heart Failure
Arrhythmia
Sudden Cardiac Death
Re-operations/interventions
Pregnancy

24. Guidelines, whenever possible, “evidence based”
Total of 647 references used
Expert consensus used when lacking evidence

25. Seen Regularly in ACHD clinic
Single Ventricle
Cyanotic
Transposition
Truncus Arteriosus
Conduits

26. Periodic ACHD follow up
Tetralogy of Fallot
Aortic Coarctation
Complicated VSD
Complicated ASD
Anom Pulm Veins
Ebstein’s anomaly

27. Non-routine ACHD patients

28. Tetralogy of Fallot Repairs in 1950s Improved survival
Longterm issuses
Heart failure
Arrhythmia
Pulmonary Valve replacement
Sudden Cardiac Death

29. Aortic Coarctation Hypertension Re-coarctation Aneurysm
Cerebral Aneurysm
Early CAD
Premature Death

30. D-Transposition

31. Atrial Switch Repair of DTGA
Mustard/Senning
1960s
Arrhythmia
Systemic RV failure

32. Arterial Switch for DTGA
Jatene
1980s
Heart Failure
PA stenosis
Aortic root dilation
Coronary ischemia

33. Hypoplastic Left Heart

34. Fontan Palliation—Single V

35. Fontan Issues….. 70% survival to adulthood Surgery in 1970s
DILV
Tricuspid Atresia
Norwood procedure
HLHS, s
70% survival to adulthood
Arrhythmia
Systolic failure
“Fontan” failure
PLE
Liver Failure/HCC
Renal Failure
Cyanosis

36. PREGNANCY

37. Pregnancy in ACHD
With more survivors and less morbidity more ability to make babies
Most woman with CHD can carry a successful pregnancy
Risk of CHD Recurrence
On average 5-8%
Up to 50%
Cardiac disease is rapidly becoming the #1 cause of peri-partum mortality

38. Normal Physiologic Changes
Reduced Systemic Vascular Resistance
BP falls early in gestation, 10mmHg below baseline by 2nd trimester
30-50% increase in intravascular volume & cardiac output
Increased lower extremity venous pressure
Fluctuation in cardiac output during labor & delivery
Increased risk of thromboembolism
Reduction in SVR can make right to left shunting worse
Patients with ventricular dysfunction or fixed Left sided obstructive lesions (like Aortic Stenosis) can have decreased cardiac output
Fluctuation is seen due to contraction (squeezing of blood/plasma out of uterus). Hemorrhage can compound the problem in preload dependent heart lesions
One series of nearly 700 patients had a 2% incidence of VTE in CHD patients (expected rate is )

39. Hemodynamic Changes Summary
Kaleschke & Baumgartner. Heart 2011.

40. For Example….

41. With decreased SVR/BP…

42. Eisenmenger Syndrome
Long standing left to right shunt (usually thru VSD) that has become right to left shunt due to development of Pulmonary Hypertension.
Contra-indication to pregnancy
50% mortality

43. Another example

44. “Flood behind the Dam”

45. Heart Failure Example

46. “Pump Failure”

47. ACHD in Peoria One of the 20 busiest clinics in US
Over 1400 visits per year
Formal transition program from Pediatric to Adult Congenital Team
~40% of patient visits at Congenital Heart Center are adults

48. THANK YOU!
QUESTIONS?