congenital heart lesions dominic blurton md pca pediatric cardiology
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Congenital Heart Lesions
Dominic Blurton MDPCA Pediatric Cardiology
OutlineNormal anatomy
1.L -> R shunt2.Left side obstruction3.Cyanotic heart lesions• Right side obstruction and R -> L shunt• Transposition4.Mixing Lesions
Surgical therapy
Pulmonary Artery
Right Atrium
Right Ventricle
Left Ventricle
Aorta
Left Atrium
Ductus Arteriosus
Patent Foramen Ovale
Key Points
• Blood flows to the path of least resistance
• Pulmonary resistance < systemic resistance
• All newborns have connections o PDAo 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
OutlineNormal anatomyL -> R shuntLeft side obstructionCyanotic heart lesions• Right side obstruction and R -> L shunt• TranspositionMixing LesionsSurgical 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)
OutlineNormal anatomyL -> R shuntLeft side obstructionCyanotic heart lesions• Right side obstruction and R -> L shunt• TranspositionMixing LesionsSurgical 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
OutlineNormal anatomyL -> R shuntLeft side obstructionCyanotic heart lesions• Right side obstruction & R -> L shunt• TranspositionMixing LesionsSurgical therapy
Cyanotic lesions
• Connection - right and left sides • AND right side obstruction• Decreased pulmonary blood flow
OR• Separated systems
Cyanotic lesions
• Right side obstructionso Tricuspid obstructiono Pulmonary obstructiono Tetralogy of Fallot
• Separate systemso Transposition of the great vessels
OutlineNormal anatomyL -> R shuntLeft side obstructionCyanotic heart lesions• Right side obstruction & R -> L shunt• TranspositionMixing LesionsSurgical therapy
Mixing lesions
• Very large intra or extracardiac connection
• Key points-o What goes into the lungs comes out of the
lungs = red o What goes into the body comes out of the
body = blue • May have right side obstruction
Mixing Lesions
• Single ventricleo Double inlet left ventricle (DILV)o Double outlet right ventricle (DORV)o Primitive ventricleo Hypoplastic right or left ventricle
• Total anomalous pulmonary venous return (TAPVR)
• Truncus arteriosus
OutlineNormal anatomyL -> R shuntLeft side obstructionCyanotic heart lesions• Right side obstruction & R -> L shunt• TranspositionMixing LesionsSurgical therapy
Surgical therapy
• Repair vs. palliation• Palliating a single ventricle - Example:
HLHS o Stage I: Norwood and BT shunto Stage II: Glenn shunto 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)
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