Treatment
Neonates:
- Prostaglandin E2 to maintain ductal patency and pulmonary blood flow
- Blalock-Taussig shunt to increase pulmonary flow
Older children:
- Glenn anastomosis - right superior vena cava to right pulmonary artery - to provide deoxygenated blood to the lungs Later surgical management:
- Fontan operation - anastomosis of right atrium to pulmonary artery either directly or via a conduit
- If right ventricular size permits a modified Fontan procedure in which a valve containing conduit is placed between the right atrium and ventricle producing a four chamber four valve heart
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