Background: Anomalous aortic origin of a coronary artery (AAOCA) encompasses a wide morphologic spectrum, which has impeded consensus regarding indications for the diverse repair strategies. We constructed a profile of current surgical techniques and explore their application to morphologic variants. Methods: Patients <30 years old (n = 113) with isolated AAOCA who underwent operations at 29 Congenital Heart Surgeons Society (CHSS) institutions from 1998 to 2012 were identified from the CHSS AAOCA Registry. Operative findings were related to surgical techniques at index repairs by cross-tabulation. Results: Anomalous origin of the left main or left anterior descending coronary artery was present in 33 (29%) patients and of the right coronary artery in 78 (69%) patients; 2 arteries originated directly above the commissure between the left and right sinuses. There were 101 (89%) interarterial and intramural (IA/IM) arteries, 10 (9%) were interarterial but not intramural (IA/NIM) and 2 (2%) were neither interarterial nor intramural. Intramural arteries were unroofed in 100 (88%) operations, usually with intimal tacking after incision (n = 47) or excision (n = 25) of the common wall. Coronary reimplantation (n = 11), pulmonary artery relocation (n = 7; 5 for IA/NIM), simple ostioplasty (without unroofing; n = 3), coronary artery bypass grafting (n = 2), and ostial window (n = 1) were less common. In 37 (33%) operations, a valvar commissure was taken down; 33 were resuspended. Conclusion: Current surgical repair of AAOCA is individualized to morphology, particularly the presence of intramural and/or interarterial segments. This report is foundational for future planned CHSS studies that will examine interventional and noninterventional outcomes and ultimately guide management of AAOCA.

Early survival after the Fontan operation has improved significantly leading to a lifetime of Fontan circulation for patients. There is a natural progression of pulmonary artery pressure during the human lifetime that is easily tolerated in two-ventricle physiology but is deleterious for single-ventricle physiology. Obstructions in the venous to pulmonary artery pathway and pulmonary venous return or ventricular outflow, atrioventricular valve dysfunction (regurgitation), pulmonary arteriovenous malformations, venous thrombosis, and the development of atrial arrhythmias can lead to reduced functional status. These complications have been demonstrated in older patients with largely atriopulmonary connections owing to time accrual and unfavorable flow characteristics. Various surgical and catheter interventions aimed at reducing morbidity and mortality have been attempted to treat these identifiable abnormalities. The purpose of this article is to review the extant literature on Fontan Conversion and to assess the lessons learned over 20 years.