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)