A case with heterotaxy reaching the Fontan procedure after unifocalizations

One patient with major aorto-pulmonary collateral arteries and heterotaxy underwent staged unifocalizations, and eventually the staged Fontan completion. Subsequent to the bidirectional Glenn procedure, the left pulmonary artery was once thrombozed, but successfully treated. The progressively regurgitant common atrioventricular valve needed repair twice during the course. Pulmonary arterial pressure was 11 mm Hg 1 year after the Fontan procedure.