1. Congenital Heart Lesions
Dominic Blurton MD
PCA Pediatric Cardiology

2. Outline Normal anatomy 1.L -> R shunt 2.Left side obstruction
3.Cyanotic heart lesions
Right side obstruction and R -> L shunt
Transposition
4.Mixing Lesions
Surgical therapy

3. Ductus Arteriosus Left Atrium Right Atrium Left Ventricle
Aorta
Pulmonary
Artery
Left Atrium
Patent
Foramen
Ovale
Heart is a pump
There are two systems: right and left
Left ventricle
Aorta – ascending, arch, and descending
Ductus arteriosus –
Periductal location
Right Atrium
Left Ventricle
Right Ventricle

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

5. Physiological classification of defects
1.L -> R shunt
2.Left side obstruction
3.Cyanotic heart lesions
Right side obstruction and R -> L shunt
Transposition
4.Mixing Lesions

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

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

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

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

15. Not enough blood to the body Hypo-perfusion, acidosis, shock
Left side obstruction
Not enough blood to the body
Hypo-perfusion, acidosis, shock

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

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

23. Cyanotic lesions Connection - right and left sides
AND right side obstruction
Decreased pulmonary blood flow OR
Separated systems

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

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

35. Mixing lesions Very large intra or extracardiac 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

36. 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

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

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

41. Hypoplastic Left Heart Syndrome

42. Stage I: Norwood + BT shunt

43. Stage II: Glenn shunt

44. Stage III: Fontan

45. Norwood RMBTS

46. Norwood RMBTS

47. Norwood RMBTS

48. Norwood Sano

49. Norwood Sano

50. RMBTS

51. Glenn for HLHS

52. Right Bidirectional Glenn

55. Single Ventricle Palliation
Neonatal sx: Norwood versus BT shunt alone
6 months age: Glenn
3 years age : Fontan
(most variability of age (1 year to 5 years)

56. Complete Repair

57. What is a complete repair
Is the heart now normal?
Are there residual lesions?
Will further touch up surgery be needed?

61. Arterial Switch

62. Arterial Switch (ASO, Jatene)

64. Konno (LVOT enlargement)