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” Wide fixed S2
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 Large 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 Large 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 Large defect Conservative management
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 Older infant
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 Older infant 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 Infant Older child
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 Neurologic
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. Cyanotic Congenital Heart Disease
Increased Pulmonary Blood Flow
TGA
TAPVD
Truncus arteriosus

43. d-Transposition

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

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

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

47. 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

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

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

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

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

52. Balloon Atrial Septostomy

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

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

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

59. TAPVD History Not a PGE dependent lesion
Presentation depends on presence or absence of obstruction to venous return
Infradiaphragmatic
Almost always obstructed
Cyanosis and respiratory distress shortly after birth
Cardiac or supracardiac
Rarely obstructed
Can present like big ASD with cyanosis
Not a PGE dependent lesion

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

61. TAPVD

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

63. Truncus arteriosus
1. common, single outflow tract with pulmonary arteries originating from the ascending aorta
2. abnormal truncal valve
3. large VSD
4. not a PGE dependent lesion

64. Cyanotic Congenital Heart Disease
Decreased Pulmonary Blood Flow

65. Cyanotic Congenital Heart Disease
Decreased Pulmonary Blood Flow
Tetralogy of Fallot/Pulmonary Atresia
Tricuspid Atresia
Critical pulmonary valve stenosis

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

67. Cyanotic Congenital Heart Disease
Tetralogy of Fallot
1. Pulmonary stenosis
2. Overriding aorta
3. RVH
4. VSD
Generally not a PGE dependent lesion

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

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

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

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

72. Tetralogy of Fallot Hypercyanotic Spells (“Tet” Spells)
Stress leading to fall in P02
Tachycardia and Hyperventilation
Increased R to L shunt
Increased Return of deeply desaturated venous blood

73. Tetralogy of Fallot Hypercyanotic Spells (“Tet” Spells Treatment
Tuck knees to chest
Reduces venous return by compressing femoral veins
Increases systemic vascular resistance
In hospital O2
Phenylephrine
Morphine
IV beta blocker

74. Tetralogy of Fallot

75. Tetralogy of Fallot
Decreased Pulmonary Blood Flow

76. Pulmonary atresia/VSD
Tetralogy of Fallot with atretic pulmonary valve
Variable pulmonary artery anatomy
Generally a PGE dependent lesion

77. Critical pulmonary stenosis
Severe pulmonary stenosis with inadequate pulmonary flow
Pulmonary atresia/intact ventricular septum
PGE dependent lesion

78. Tricuspid atresia Generally a PGE dependent lesion
2. severely hypoplastic RV
3. VSD
4. ASD – large
5. pulmonary stenosis
Variable
Generally a PGE dependent lesion

79. Cyanotic Heart Disease
Decreased blood flow due to RVOT obstruction may require augmentation of pulmonary blood flow via creation of a surgical systemic to pulmonary shunt
Blalock-Taussig Shunt (BTS)

80. Case 1 (continued)
BTS 80

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

83. Critical Left-Sided Obstruction
Neonatal presentation
Coarctation
Critical aortic stenosis
Hypoplastic left heart syndrome
Cardiogenic shock
PGE dependent lesion

84. Left-sided Obstruction
Coarctation of the aorta
Critical narrowing of the “juxtaductal” aorta
Blood cannot get past the obstruction
SHOCK

85. Coarctation
Characterized by weak or absent pulses particularly in the lower limbs
Initiation of PGE lifesaving
‘splitting’ of saturations seen in critical narrowings with patency of ductus arteriosus ie: normal saturation in right arm and lower saturation in the lower limbs due to right to left shunting across the PDA

86. Coarctation - treatment
Surgical correction following initiation of PGE and stabilization

87. Left-Sided Obstruction
Critical Aortic Stenosis
CRITICAL
Inadequate forward
flow to maintain
cardiac output
SHOCK

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

89. Left Ventricular Outflow Tract Obstruction
Hypoplastic Left Heart Syndrome (HLHS)
1. Mitral atresia
2. Aortic atresia
3. Hypoplastic left ventricle
4. Hypoplastic ascending aorta
PDA is the only source of systemic blood flow
PGE dependent lesion

90. HLHS Initially cyanotic With closure of the PDA SHOCK PGE
Tachycardia, tachypnea, low blood pressure, weak pulses, poor perfusion, cyanotic/grey colour
PGE

91. Hypoplastic left heart
Management
Prostaglandins
Norwood procedure
Heart Transplant

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

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

94. Kawasaki Syndrome Associated Findings Sterile pyuria
Hydrops of the gallbladder
Irritability

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

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

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

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

100. Questions?