A four-year-old girl with heterotaxy syndrome and right atrial isomerism, right dominant atrioventricular septal defect, pulmonary atresia, and right aortic arch status post single ventricle palliative surgery with a bidirectional Glenn presented for the Fontan procedure. She had a tri-leaflet aortic valve and no history of aortic arch reconstruction. Although she tolerated her Fontan procedure well, her postoperative course was complicated by ventricular tachycardia and severely depressed right…