ABSTRACT
Patients with polysplenia syndrome and single ventricle anatomy may have interrupted inferior vena cava (IVC) with azygos or hemiazygos continuation to the superior vena cava (SVC). AP Fontan with a classic Glenn shunt predisposes to pulmonary arteriovenous fistulas when the hepatic venous return is excluded from part of the pulmonary circulation. This can be reversed with subsequent pathway revision (reconnecting divided pulmonary arteries and TCPC) to include hepatic venous drainage.
