Congenital Heart Lesions

1. Congenital Heart Lesions

2. Outline Normal anatomy L -> R shunt Left side obstruction Cyanotic heart lesions • Right side obstruction and R -> L shunt • Transposition Mixing Lesions Surgical therapy

3. Ductus Arteriosus Aorta Pulmonary Artery Left Atrium Patent Foramen Ovale Right Atrium LeftVentricle Right Ventricle

4. Key Points • Blood flows to the path of least resistance • Pulmonary resistance < systemic resistance • All newborns have connections • PDA • PFO

5. Outline Normal anatomy L -> R shunt Left side obstruction Cyanotic heart lesions • Right side obstruction and R -> L shunt • Transposition Mixing Lesions Surgical therapy

6. Left to right shunting • Right and left side connected • Increased (too much) pulmonary blood flow • Respiratory distress/ CHF

7. Left to right shunt lesions • Ventricular septal defect (VSD) • Atrial septal defect (ASD) • AV canal • Patent ductus arteriosus (PDA)

12. Diagnostic tools • CXR-- “wet lungs” with cardiomegaly • EKG-- may have RVH, IRBBB (ASD), abnormal “NW” axis (AV canal), BVH (VSD) • ABG-- high CO2 late finding; PO2 in 100% not very useful; no acidosis

13. Outline Normal anatomy L -> R shunt Left side obstruction Cyanotic heart lesions • Right side obstruction and R -> L shunt • Transposition Mixing Lesions Surgical therapy

14. Left side obstruction • Not enough blood to the body • Hypo-perfusion, acidosis, shock • +/- connection between right and left

15. Left side obstructive lesions • Mitral valve obstruction • Aortic valve obstruction • Coarctation of the aorta • Everything obstructed • Hypoplastic left heart syndrome

19. Diagnostic tools • CXR- may be normal or “wet” • EKG- often misleading; neonate will not have LVH you would expect from an older person with AS or coarct (and hypoplast will have left forces) • ABG- may present with profound metabolic acidosis, low CO2 (hyperventilating), PO2 may be lo or hi

20. Outline Normal anatomy L -> R shunt Left side obstruction Cyanotic heart lesions • Right side obstruction & R -> L shunt • Transposition Mixing Lesions Surgical therapy

21. Cyanotic lesions • Connection - right and left sides • AND right side obstruction • Decreased pulmonary blood flow OR • Separated systems • Normal or increased pulmonary blood flow

22. Cyanotic lesions • Right side obstructions • Tricuspid obstruction • Pulmonary obstruction • Tetralogy of Fallot • Separate systems • Transposition of the great vessels

27. Diagnostic tools • CXR- classically, “black lung fields” with “boot” (TOF) or narrow mediastinum (TGA) • EKG- very often normal, except tricuspid atresia classically “northeast” • ABG- these are the kids who fail the hyperoxia challenge

29. Outline Normal anatomy L -> R shunt Left side obstruction Cyanotic heart lesions • Right side obstruction & R -> L shunt • Transposition Mixing Lesions Surgical therapy

30. When is “blue” O.K.?

31. Mixing lesions • Very large connection • Key points- • What goes into the lungs comes out of the lungs = red • What goes into the body comes out of the body = blue • May have right side obstruction

32. Mixing Lesions • Single ventricle • Double inlet left ventricle (DILV) • Double outlet right ventricle (DORV) • Primitive ventricle • Hypoplastic right or left ventricle • Total anomalous pulmonary venous return (TAPVR) • Truncus arteriosus

36. Outline Normal anatomy L -> R shunt Left side obstruction Cyanotic heart lesions • Right side obstruction & R -> L shunt • Transposition Mixing Lesions Surgical therapy

37. Surgical therapy • Repair vs. palliation • Palliating a single ventricle - Example: HLHS • Stage I: Norwood and BT shunt • Stage II: Glenn shunt • Stage III: Fontan

38. Hypoplastic Left Heart Syndrome

39. Stage I: Norwood + BT shunt

40. Stage II: Glenn shunt

41. Stage III: Fontan

42. Take-home • Congenital heart disease is not about murmurs • Tachypnea, cyanosis, “shock” should all raise red flags • Exam, CXR,EKG,Sats, ABG are as important as the echo!