2. Paediatric Cardiology: An Outline of Congenital Heart Disease Dr. H.C. Rosenberg hrosenberg@cheo.on.ca

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

4. An Outline of Congenital Heart Disease u Pink (Acyanotic) u Blue (Cyanotic)

5. u Resistance= ?

6. Acyanotic Congenital Heart Disease u Normal Pulmonary Blood Flow u ↑ Pulmonary Blood Flow

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

8. Acyanotic Congenital Heart Disease u ↑ Pulmonary Blood Flow

9. Shunt Lesions Atrial Level Shunt

10. ASD Physiology u Left to Right shunt because of greater compliance of right ventricle u Loads right ventricle and right atrium u Increased pulmonary blood flow at normal pressure u Low resistance

11. ASD History u Usually asymptomatic in childhood u Occasionally frequent respiratory tract infections u Presentation with murmur as pre-schooler or older

12. ASD Physical Examination u Right ventricular “lift” u Wide fixed S2 u Blowing SEM in pulmonic area

13. ASD

14. ASD

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

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

17. ASD

18. Shunt Lesions Ventricular Level Shunt

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

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

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

22. VSD BVH

23. VSD

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

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

26. Shunt Lesions Great Artery Level Shunt

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

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

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

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

31. Truncus Arterisosus

32. Cyanotic Congenital Heart Disease u “Blue” blood (deoxygenated hemoglobin” enters the arterial circulation u Systemic oxygen saturation is reduced u Cyanosis may or may not be clinically evident

33. Causes of Cyanosis u Respiratory u Cardiac u Hematologic u Polycythemia u Hemoglobins with decreased affinity u Neurologic u Decreased Respiratory drive

34. Cyanosis u Respiratory u Cardiac u Hyperoxic test u Place infant in 100% 02 u Lung disease should respond to 02 u Failure of saturation to rise to > 85% suggest cardiac disease

35. Cyanotic Congenital Heart Disease u ↓Pulmonary Blood Flow u ↑Pulmonary Blood Flow

36. Cyanotic Congenital Heart Disease u Decreased Pulmonary Blood Flow

37. Cyanotic Congenital Heart Disease u Decreased Pulmonary Blood Flow u Tetralogy of Fallot u Pulmonary Atresia

38. Cyanotic Congenital Heart Disease - ↓ Pulmonary Flow = RVOT Obstruction + Shunt

39. Tetralogy of Fallot u VSD u Over-riding aorta u Pulmonary stenosis u RVH

40. Tetralogy of Fallot

41. History u Presentation depends on severity of PS u Severe stenosis u Cyanosis shortly after birth (as duct closes) u Mild stenosis u May present as heart murmur (from shortly after birth)

42. Tetralogy of Fallot Physical Examination u Variable cyanosis (remember the 50g/l rule) u Right ventricular “tap” u Decreased P2 +/- ejection click u “Tearing” SEM

43. Tetralogy of Fallot Management u Outside the newborn period, surgical repair if symptomatic u Elective repair at 6 months u Role for beta blockers to palliate hypercyanotic spells

44. Tetralogy of Fallot Hypercyanotic Spells (“Tet” Spells) u Episodes of profound cyanosis u Most frequently after waking up or exercise

45. Tetralogy of Fallot Hypercyanotic Spells (“Tet” Spells) Fall in P0 2 Hyperventilation Increased Return of deeply desaturated venous blood Increased R to L shunt

46. Tetralogy of Fallot Hypercyanotic Spells (“Tet” Spells u Treatment u Tuck knees to chest (pinches off femoral veins) u In hospital u O2 u Bicarbonate u Phenylephrine u Morphine u IV beta blocker

47. Tetralogy of Fallot

48. u Decreased Pulmonary Blood Flow

49. Duct Dependent Congenital Heart Disease  Which of the following are examples of duct dependent CHD? 1. Pulmonary atresia 2. Patent ductus arteriosus 3. Transposition of the great arteries

50. Cyanotic Congenital Heart Disease With ↑Pulmonary Blood Flow

51. u Transposition of the great arteries u Total anomalous pulmonary venous drainage

52. d-Transposition

53. Normal Heart BodyRARVPA LALVAOLungs Circulation is in “series”

54. d-Transposition u Circulation is in “parallel” u Need for mixing

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

56. Tetralogy of Fallot Physical Examination u Profound cyanosis u Right ventricular “tap” u Loud single S2 u Little or no murmur

57. Tetralogy of Fallot Management u Prostaglandins to maintain mixing u Balloon atrial septostomy u Arterial switch repair in first week

58. Total Anomalous Pulmonary Venous Return Pulmonary veins fail to connect to left atrium Pulmonary veins communicate with systemic vein

59. Total Anomalous Pulmonary Venous Return - Supracardiac Pulmonary veins fail to connect to left atrium Pulmonary veins communicate with systemic vein

60. Total Anomalous Pulmonary Venous Return - Infracardiac Pulmonary veins fail to connect to left atrium Pulmonary veins communicate with systemic vein

61. TAPVD History u Presentation depends on presence or absence of obstruction to venous return u Infradiaphragmatic u Almost always obstructed u Cyanosis and respiratory distress shortly after birth u Cardiac or supracardiac u Rarely obstructed u Can present like big ASD

62. TAPVD Physical Examination u Variable cyanosis (again depends on obstruction) u Right ventricular “tap” u Wide split S2 u Blowing systolic ejection murmur

63. TAPVD

64. TAPVD Management u If severe cyanosis in newborn u Emergency surgical repair u Unobstructed u Semi-elective surgical repair when discovered

65. Coarctation of the aorta

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

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

68. Coarctation Management u Newborn with CHF u Emergency surgical repair u Infant u Semi-elective repair in uncontrolled hypertension u Older child u Balloon arterioplasty u Surgery on occasion u Failure to repair prior to adolescence recipe for life long hypertension!

69. “Grey” Heart Disease u Critical LVOT obstruction

70. Left Ventricular Outflow Tract Obstruction u Critical Aortic Stenosis u “Critical” shock

71. Critical Aortic Stenosis Management u Prostaglandins to provide source of systemic blood flow u Balloon valvuloplasty u Rarely surgery

72. Hypoplastic Left Heart Syndrome u “Duct dependent “ congenital heart disease u Ductus arteriosus is the only source of systemic blood flow

73. Hypoplastic left heart Management u Prostaglandins u Norwood procedure

74. Kawasaki Syndrome u Small artery arteritis u Coronary arteries most seriously effected u Dilatation/aneurysms progressing to (normal) stenosis

75. Kawasaki Syndrome u 5 days of fever plus 4 of  Rash  Cervical lymphadenopathy (at least 1.5 cm in diameter)  Bilateral conjuctival injection  Oral mucosal changes u Peripheral extremity changes u Swelling u Peeling (often late)

76. Kawasaki Syndrome u Associated Findings u Sterile pyuria u Hydrops of the gallbladder u Irritability!!!

77. Kawasaki Syndrome u Epidemiology u Generally children < 5 years u Male > Female u Asian > Black > White

78. Kawasaki Syndrome u Management u Gamma globulin 2g/kg u 80 mg/kg ASA until afebrile then 5 mg/kg for 6 weeks

79. Innocent Murmurs u Characteristics u Always Grade III or less u Always systolic (or continuous) u Blowing or musical quality u Not best heard in back

80. Innocent Murmurs u Types u Still’s u Vibratory SEM best heard mid-left sternal border u Pulmonary Flow murmur u Blowing SEM best heard in PA u Venous Hum u Continuous murmur best heard in R infraclavicular u Decreases lying flat or occlusion of neck veins u Physiologic peripheral pulmonary artery stenosis u Blowing SEM best heard in PA radiating out to both axillae

81. Questions?