Without surgical palliation, single ventricle heart defects are uniformly fatal. A three-staged surgical repair is typically performed on these patients, who are otherwise severely cyanotic. In the third stage, the Fontan procedure, the inferior vena cava (IVC) is connected to the pulmonary arteries (PAs) via a lateral tunnel or extracardiac conduit. Following Fontan completion, deoxygenated blood from the upper and lower body is redirected to the PAs, bypassing the heart.

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