Adult Congenital Heart Disease

1. Adult Congenital Heart Disease Carlos Macias, MD Arizona Pediatric Cardiology Consultants

2. Congenital Heart Disease • An abnormality of the heart that is present at birth. It may be hereditary or due to some influence occurring during pregnancy.

3. Congenital Heart Disease • 95% has no identifiable cause. • Ranges in severity from fairly simple to and curable to very complex and potentially lethal.

4. Congenital Heart Disease • The most common birth defect. • Occurs in @ 1/100 births

5. History of Congenital Heart Surgery • 1940’s-1950’s Maude Abbott and Helen Taussig defined the anatomy and physiology of Congenital Heart Disease. • Early 1950’s, first closed heart surgery using bathtub hypothermia. • 1953, First attempts at heart “bypass” surgery. • 1955, first bypass surgery at Mayo Clinic.

6. Congenital Heart DefectsPrevalence • 32,000 new cases of CHD / year in the U.S. • 1.5 million, world wide. • 20,000 open-heart operations / year in U.S. • > 85% reaching adulthood • Approx.. 1,000,000 adults with CHD in U.S. • More adults than children with CHD

7. Changes in Technology • Preoperative diagnosis • Intraoperative care (cardiopulmonary bypass). • Surgical technique • Postoperative care

8. ACHD • For all but the most simple defects, surgical and catheter procedures are palliative rather that curative.

9. Acyanotic CHD(left to right shunt) • Atrial septal defect (ASD) • Ventricular septal defect (VSD) • Endocardial cushion defect • Patent ductus arteriosus (PDA)

10. Cyanotic CHD(right to left shunt) • Obstruction to pulmonary blood flow • Tricuspid, pulmonary atresia • Intracardiac mixing of oxygenated and unoxygenated blood • Tetralogy of Fallot • Transposition of the great Vessels • Single ventricle

11. Obstructive Valvar Lesions • Pulmonary valve stenosis • Aortic valve stenosis. • Coarctation of the thoracic aorta

12. Fetal Circulation

13. Internal Normal Heart (Frontal View)

14. Atrial Septal Defect

15. Atrial Septal Defects(View from right side)

16. Atrial Septal DefectPatch Repair

17. Amplatzer Occlusion of Atrial Septal Defect Clockwise from above: Transcatheter delivery of Amplatzer device, which is positioned across the atrial septal defect Left: Amplatzer device in place

20. ASD, Survival Patterns • Normal, if closed before 20 years of age. • Good, but shorter than normal 25-41 yrs. • Increased morbidity and mortality after 41 yrs.

21. ASD and PHT • Occurs in two chronologically disparate age groups. • Females, late teens and 20s. • Primary PHT with coincidental ASD • Don’t close the ASD !! • Patients > 40 years of age • Mild to mod. PHT secondary to longstanding left to right shunt. • Likely to benefit from ASD closure

22. Ventricular Septal Defect VSD before surgical repair VSD after surgical repair

23. Ventricular Septal Defects(View from right side)

24. Occlusion of Intracardiac and Vascular ShuntsVentricular Septal Defect Occlusion Above: Echocardiogram of muscular VSD Upper right: Fluoro image of CardioSEAL device occlusion of a VSD. Transesophageal echo probe (TEE) and pigtail catheter in place. Lower right: Amplatzer muscular ventricular septal occluder Illustration courtesy AGA Medical Group

25. Eisenmenger Complex Ventricular Septal Defect (VSD) in conjunction with Pulmonary Vascular Obstructive Disease (PVOD)Arrow – right to left shunt

26. Atrioventricular Canal Defect- Complete

27. Atrioventricular Canal Defect - Complete Above: view of heart from above showing joined mitral and tricuspid valvesRight: ASD – atrial septal defectVSD – ventricular septal defect

28. Atrioventricular Canal Defect- Complete

29. Atrioventricular Canal Defect - Partial

30. Repair of Atrioventricular Canal Defect - Complete

31. Patent Ductus Arteriosus (PDA)

32. Patent Ductus Arteriosus Division and Ligation

33. Occlusion of Intracardiac and Vascular ShuntsCoil embolization of PDA Left, top: Catheter crosses the PDA from the aortic side and delivers a coil. Left, bottom: Withdrawal of catheter, leaving coil in PDA

34. AorticStenosisA - Supravalvar B – ValvarC - Subvalvar

35. Severe Aortic Stenosis in an infant RV RA LA 1) Valvular Stenosis with a bicuspid aortic valve

36. Aortic Stenosis - Balloon Valvuloplasty

37. Repair of Aortic Stenosis Ross Procedure Konno Procedure

38. Marfan’sSyndrome1 – enlarged ascending aorta2 – stretched aortic valve3 – mitral valve prolapseSV – Sinuses of Valsalva

39. Pulmonary StenosisA – supravalvularB – valvularC - subvalvular

40. ValvuloplastyPulmonary Balloon Valvuloplasty Left: Arrow indicates stenotic pulmonary valve

41. Coarctation of the AortaSA – subclavian artery COA – coarctationAV - aortic valve

42. Coarctation of the Aortawith severe aortic stenosis in an infant 1. Coarctation of the aorta, distal to the left subclavian artery 2. Severe aortic stenosis

43. Coarctation Repair Excision with End to End Anastamosis

44. Coarctation - Subclavian Flap Repair

45. AngioplastyAortic Coarctation Angioplasty Illustrations showing (left) uninflated and (right) inflated angioplasty balloon positioned within coarctation of the descending aorta

46. Intravascular StentsCoarctation of the Aorta Left: uninflated angioplasty balloon and stent within coarctation Middle: expansion of balloon and stent Right: deflation of balloon leaving stent wide open

47. Transposition of the Great Arteries – L TypeMV – mitral valveTV – tricuspid valve

48. Ebstein’s AnomalyASD – atrial septal defect1 – atrialization of the right ventricle

50. Transposition of the Great Arteries – D Type