Paediatric Cardiology: Congenital Heart Disease and Clinical Problems

1. Paediatric Cardiology:Congenital Heart Disease and Clinical Problems Dr. Suzie Lee Pediatric Cardiologist Assistant Professor, University of Ottawa

2. Objectives • To provide an outline of congenital heart disease • List criteria for Kawasaki syndrome • Describe the common innocent murmurs of childhood

3. An Outline of Congenital Heart Disease • Pink (Acyanotic) • Blue (Cyanotic) • Critical outflow tract obstruction

4. Acyanotic Congenital Heart Disease • Normal Pulmonary Blood Flow • ↑Pulmonary Blood Flow

5. Acyanotic Congenital Heart Disease • Normal Pulmonary Blood Flow • Valve Lesions • Not fundamentally different from adults

6. Acyanotic Congenital Heart Disease • ↑Pulmonary Blood Flow

7. Shunt Lesions Atrial Level Shunt

8. ASD Physiology • Left to Right shunt because of greater compliance of right ventricle • Loads right ventricle and right atrium • Increased pulmonary blood flow at normal pressure

9. ASD History • Usually asymptomatic in childhood • Occasionally frequent respiratory tract infections • Presentation with murmur in childhood

10. ASD Physical Examination • Right ventricular “lift” • Atrial level shunts result in right-sided volume overload • Wide fixed S2 • Blowing SEM in pulmonic area • Murmur due to increased flow across the pulmonary

11. ASD

12. ASD

13. ASD Natural History • Generally do well through childhood • Major complication atrial fibrillation • Can develop pulmonary hypertension / RV failure but not before third or fourth decade of life

14. ASD Management • Device closure around three years of age or when found • Surgery for very large defects or outside fossa ovalis (eg. sinus venosus defect)

15. ASD

18. Shunt Lesions Ventricular Level Shunt

19. VSD Physiology • Left to Right shunt from high pressure left ventricle to low pressure right ventricle • Loads left atrium and left ventricle (right ventricle may see pressure load)

20. VSD History • Small defects • Presentation with murmur in newborn period • Large defects • Failure to thrive (6 wks to 3 months) • Tachypnea, poor feeding, diaphoresis

21. VSD Physical Examination • Active left ventricle • Small defect • Pansystolic murmur, normal split S2 • Large defect • SEM, narrow split S2, diastolic murmur at apex from high flow across mitral valve

22. VSD

23. VSD

24. VSD Natural History • Small defect • Often close • No real significance beyond endocarditis risk • Large defect • Failure to thrive • Progression to pulmonary hypertension as early as 1 year

25. VSD Management • Small defect • Conservative management • Large defect • Semi-elective closure if growth failure or evidence of increased pulmonary hypertension • Occasionally elective closure if persistent cardiomegaly beyond 3 years of age

26. Shunt Lesions Great Artery Level Shunt

27. PDA Physiology • Left to Right shunt from high pressure aorta to low pressure pulmonary artery • Loads left atrium and left ventricle (right ventricle may see pressure load)

28. PDA History • Premature duct • Failure to wean from ventilator +/- murmur • Older infant • Usually murmur from early infancy • Occasionally signs of heart failure

29. PDA Physical Examination • Active left ventricle • Hyperdynamic pulses • Premature duct • SEM with diastolic spill • Older infant • Continuous murmur

30. PDA Management • Premature Duct • Trial of indomethacin • Surgical ligation • Older infant • Leave till 1 year of age unless symptomatic • Coil / device closure • Rarely surgical ligation

31. Coarctation • Obstruction of the aortic arch • Classically juxtaductal, although may occur anywhere along the aorta • May develop over time • Femoral pulses should be checked routinely throughout childhood

32. Coarctation of the Aorta History • Presentation varies with severity • Severe coarct • Failure (shock) in early infancy • Mild coarct • Murmur (in back) • Hypertension

33. Coarctation Physical Examination • Absent femoral pulses • Arm leg gradient +/- hypertension • Left ventricular “tap” • Bruit over back

34. Coarctation Management • Newborn with CHF • Emergency surgical repair • Infant • Semi-elective repair in uncontrolled hypertension • Older child • Balloon arterioplasty +/- stenting • Surgery on occasion • Failure to repair prior to adolescence recipe for life long hypertension

37. Cyanotic Congenital Heart Disease • “Blue” blood (deoxygenated hemoglobin) enters the arterial circulation • Systemic oxygen saturation is reduced • Cyanosis may or may not be clinically evident • 5g% deoxygenated HgB

38. Causes of Cyanosis • Respiratory • Cardiac • Hematologic • Polycythemia • Hemoglobins with decreased affinity • Neurologic • Decreased Respiratory drive

39. Cyanosis • Respiratory • Cardiac • Hyperoxic test – response to 100% O2 • Lung disease should respond to 02 • PO2 should rise to greater than 150 mmHg

40. Cyanotic Congenital Heart Disease • Increased pulmonary blood flow • Truncus arteriosus • Transposition of the great arteries • Total anomolous pulmonary venous return • Decreased pulmonary blood flow • Tetralogy of Fallot/pulmonary atresia • Tricuspid atresia • Critical pulmonary stenosis

41. Cyanotic Congenital Heart Disease ↑Pulmonary Blood Flow

42. d-Transposition

43. Normal Heart Body RA RV PA AO LV LA Lungs Circulation is in “series”

44. d-Transposition Circulation is in “parallel” Body RA RV Ao Lungs LA LV PA

45. d-Transposition • Circulation is in “parallel” • Need for mixing

46. TGA Must bring oygenated blood into the systemic circulation Great artery level shunt - PDA Atrial level shunt – PFO Prostaglandin E1 (PGE) Re-opens and maintains patency of the ductus arteriosus Balloon atrial septostomy (BAS) Increase intracardiac shunting across the atrial septum

47. d-Transposition Body RA RV Ao PFO BAS PDA PGE Lungs LA LV PA

48. Transposition History • Presentation • Profound cyanosis shortly after birth (as duct closes) • Minimal or no murmur

49. TGA Physical Examination • Profound cyanosis • Right ventricular “tap” • Loud single S2 • Little or no murmur

50. TGA Management • Prostaglandins to maintain mixing • Balloon atrial septostomy • Arterial switch repair in first week