Paediatric Cardiology: A “review” of Congenital Heart Disease and Clinical Problems

1. Paediatric Cardiology:A “review” of 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 • Review of pediatric ECGs

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 until 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 coarctation • Failure (shock) in early infancy • Mild coarctation • 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. TGA

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

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

45. TGA • 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. TGA Body RA RV Ao PFO BAS PDA PGE Lungs LA LV PA

48. TGA History • Presentation • Profound cyanosis shortly after birth • Particularly with restrictive ASD and/or closure of the ductus arrteriosus • 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