Congenital Heart
Lesions
Dominic Blurton MD
PCA Pediatric Cardiology
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
Ductus
Arteriosus
Aorta
Pulmonary
Artery
Left Atrium
Patent
Foramen
Ovale
Right Atrium
Left
Ventricle
Right Ventricle
Key Points
• Blood flows to the path of least
resistance
• Pulmonary resistance < systemic
resistance
• All newborns have connections
o
o
PDA
PFO
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
Outline
Normal anatomy
L -> R shunt
Left side obstruction
Cyanotic heart lesions
• Right side obstruction and R -> L shunt
• Transposition
Mixing Lesions
Surgical therapy
Left to right shunting
• Right and left side connected
• Increased (too much) pulmonary blood
flow
• Respiratory distress/ CHF
Left to right shunt lesions
•
•
•
•
Ventricular septal defect (VSD)
Atrial septal defect (ASD)
AV canal
Patent ductus arteriosus (PDA)
Outline
Normal anatomy
L -> R shunt
Left side obstruction
Cyanotic heart lesions
• Right side obstruction and R -> L shunt
• Transposition
Mixing Lesions
Surgical therapy
Left side obstruction
• Not enough blood to the body
• Hypo-perfusion, acidosis, shock
Left side obstructive lesions
•
•
•
•
Mitral valve obstruction
Aortic valve obstruction
Coarctation of the aorta
Everything obstructed
o
Hypoplastic left heart syndrome
Outline
Normal anatomy
L -> R shunt
Left side obstruction
Cyanotic heart lesions
• Right side obstruction & R -> L shunt
• Transposition
Mixing Lesions
Surgical therapy
Cyanotic lesions
• Connection - right and left sides
• AND right side obstruction
• Decreased pulmonary blood flow
OR
• Separated systems
Cyanotic lesions
• Right side obstructions
o
o
o
Tricuspid obstruction
Pulmonary obstruction
Tetralogy of Fallot
• Separate systems
o
Transposition of the great vessels
Outline
Normal anatomy
L -> R shunt
Left side obstruction
Cyanotic heart lesions
• Right side obstruction & R -> L shunt
• Transposition
Mixing Lesions
Surgical therapy
Mixing lesions
• Very large intra or extracardiac
connection
• Key pointsWhat goes into the lungs comes out of the
lungs = red
o What goes into the body comes out of the
body = blue
o
• May have right side obstruction
Mixing Lesions
• Single ventricle
o
o
o
o
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
Outline
Normal anatomy
L -> R shunt
Left side obstruction
Cyanotic heart lesions
• Right side obstruction & R -> L shunt
• Transposition
Mixing Lesions
Surgical therapy
Surgical therapy
• Repair vs. palliation
• Palliating a single ventricle - Example:
HLHS
o
o
o
Stage I: Norwood and BT shunt
Stage II: Glenn shunt
Stage III: Fontan
Hypoplastic Left Heart Syndrome
Stage I: Norwood + BT shunt
Stage II: Glenn shunt
Stage III: Fontan
Norwood RMBTS
Norwood RMBTS
Norwood RMBTS
Norwood Sano
Norwood Sano
RMBTS
Glenn for HLHS
Right Bidirectional Glenn
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)
Complete Repair
What is a complete repair
• Is the heart now normal?
• Are there residual lesions?
• Will further touch up surgery be needed?
Arterial Switch
Arterial Switch (ASO, Jatene)
Konno (LVOT enlargement)