Congenital Heart Disease

1. Congenital Heart Disease Initial evaluation and stabilization Priscilla Joe, MD Children’s Hospital and Research Center Oakland

2. Initial evaluation • History • Physical exam with 4 extremity blood pressures • Pre-ductal and post-ductal oxygen saturations • Hyperoxia test • CXR • EKG • ECHO

3. Indications for fetalechocardiography Fetal risk factors associated with CHD: • Trisomies, Turner’s syndrome, abnormal karyotype • Congenital malformations: duodenal atresia, TEF, omphalocele, diaphragmatic hernia, renal dysgenesis, and hydrocephalus • Fetal arrhythmias • IUGR • Nonimmune hydrops

4. Maternal metabolic disorders or infection • Diabetes mellitus • PKU • Hyperthyroidism • Lupus, collagen vascular disease • Rubella, CMV, Coxsackie, HIV

5. Maternal risk factors associated with congenital heart disease • Congenital heart disease • Cardiac teratogen exposure • Lithium • Amphetamines • Alcohol • Anticonvulsants: phenytoin, valproic acid, carbamazepine,and trimethadione • Isotretinoin

6. Lungs vs heart:Differential cyanosis and the hyperoxia test • PaO2 <50 and SpO2 <85% pre-ductal despite 100% FiO2 -PPHN -left-heart abnormalities • Post-ductal saturation higher than pre-ductal saturation -TGA -TAPVR above diaphragm with PDA

8. Neonatal Heart Disease • Ductal dependent lesions • Congestive heart failure • Right heart obstructive lesions • Left heart obstructive lesions • Mixing lesions • Inadequate gas exchange

9. Normal heart

10. Pulmonary Hypertension

11. Pulmonary Hypertension Preductal SpO2 Postductal SpO2 PA Ao

12. Transposition of great arteries Ao PA

13. Transposition Preductal SpO2 Postductal SpO2 Ao PA

14. TAPVR Preductal SpO2 Postductal SpO2

15. CXR • Heart size • Pulmonary blood flow • Cardiac position

17. Ebstein’s anomaly

18. Cyanotic with decreased pulmonary blood flow

19. Right Sided Obstructive Lesions-Blue, but comfortable • Cyanosis • No respiratory distress • Normal pulses and perfusion • Single second heart sound (no closing sound from abnormal pulm valve) • Murmur • Moderate to marked hypoxemia • CXR: normal to large sized heart, decreased PBF

21. Tetrology of Fallot

22. Tetrology of Fallot

23. Tetrology of Fallot Infundibular septum angled anteriorly

25. Tricuspid Atresia

26. Tricuspid Atresia

27. Cyanotic with decreased pulmonary blood flow • Tetrology of Fallot • Ebsteins Anomaly • Tricuspid Atresia with PA or PS • Pulmonary atresia with intact septum • Critical pulmonic stenosis • PPHN

28. Management right sided obstructive lesions • PGE • Supplemental O2 is OK (may slightly improve pulmonary vasodilatation) • Surgical intervention

29. Left sided obstructive lesions Acute shock

30. Left sided obstructive lesions • Grey or ashen color (may not be blue) • Tachypnea • Poor perfusion • Decreased pulses/differential pulses • Single second heart sound • Murmur + gallop • Hepatomegaly • ABG: metabolic acidosis • CXR: cardiomegaly with increased PBF

31. Left sided obstructive lesions • Coarctation of aorta, interrupted aortic arch • Hypoplastic left heart syndrome • Aortic stenosis • Mitral stenosis • Total anomalous pulmonary venous return, below diaphragm

32. Hypoplastic left heart syndrome

33. Aortic stenosis

34. Hypoplastic Left Heart Syndrome • PDA supplies: • body • lungs • head • coronaries

35. Coartation of aorta

36. HLHS Treatment • Fetal diagnosis is vital to prevent end organ failure • PGE • Balance perfusion to body/coronaries/head vs lungs • Avoid oxygen, hyperventilation, pressors to limit PBF • Control ventilation; paralyze and hypoventilate • Blend in nitrogen to raise PVR and limit PBF • Surgical intervention

37. Cyanotic with increased pulmonary blood flow Inadequate mixing

38. Inadequate Mixing Lesions • Cyanosis, often profound • Mild tachypnea • Normal pulses • Single heart sound • Murmur • ABG: marked hypoxemia, + acidosis • CXR: cardiomegaly, normal or increased PBF

40. d - Transposition of the Great Vessels

41. Transposition of Great Arteries Mixing at PFO and PDA

42. Truncus arteriosus

44. Truncus arteriosus

45. Cyanotic with increased pulmonary blood flow • d-Transposition of the great vessels • Truncus arteriosus • Total anomalous pulmonary venous return, above diaphragm • Single ventricle • Endocardial cushion defect

46. Treatment of mixing lesions: TGA • PGE • Avoid too much PBF, may worsen patient • Balloon septostomy • Supplemental O2 may be helpful • Surgical repair

47. Lesions with poor gas exchange

48. Lesions with poor gas exchange • Cyanosis • Marked tachypnea (difficult to differentiate from GBS pneumonia/MAS • Perfusion fair, pulses normal • Second heart sound may be single • May or may not have a murmur • CXR: normal heart size, pulmonary congestion

49. Total anomalous pulmonary venous return

50. Supracardiac TAPVR