Cardiol Young 2006; 16 (Suppl. 3): 97–102 © Cambridge University Press ISSN 1047-9511 doi: 10.1017/S1047951106000813 A VARIETY OF SURGICAL STRATEGIES HAVE BEEN utilized in attempts to accomplish long-term haemodynamic stability in patients with car- diac anomalies characterized by discordant atrioven- tricular connections, ventricular septal defect, and severe sub-pulmonary obstruction. The majority of these patients have what is commonly referred to as congenitally corrected transposition, together with a ventricular septal defect and pulmonary stenosis or atresia, in the setting of either usual or mirror imaged arrangement of the atrial chambers and the other organs of the body. A smaller sub-group, with discordant atrioventricular connections and double outlet right ventricle, with severe sub-pulmonary obstruction or pulmonary atresia, present similar physiology, and a comparable surgical challenge. Toward the end of the decade of the 1980s, several surgical groups, 1–3 recognizing the limitations of sur- gical strategies that left the morphologically right ven- tricle as the sole systemic pumping chamber, attempted surgical repairs encompassing an atrial baf- fle procedure, either the Mustard or Senning operation, to address the physiology of discordant atrioventric- ular connections, together with a Rastelli procedure, involving construction of an interventricular baffle and placement of an extracardiac conduit, to address the problems created by, on the one hand, the discordant ventriculo-arterial connections, and on the other hand, the sub-pulmonary obstruction (Fig. 1). Ilbawi et al. 1 were the first to report a successful series of such procedures, which eventually came to be known as double switch operations. As is dis- cussed in the review of nomenclature elsewhere in this supplement, 4 this term is more precise and accu- rate when applied to the combination of an atrial baffle procedure and an arterial switch operation, as has more recently been advocated for congenitally The role of Fontan’s procedure and aortic translocation in the surgical management of patients with discordant atrioventricular connections, interventricular communication, and pulmonary stenosis or atresia Marshall L. Jacobs, 1 Glenn Pelletier, 1 Peter D. Wearden, 2 Victor O. Morell 2 1 Section of Cardiothoracic Surgery, St. Christopher’s Hospital for Children, Drexel University, Philadelphia, Pennsylvania, United States of America; 2 Section of Pediatric Cardiothoracic Surgery of the Heart, Lung and Esophageal Institute, Children’s Hospital of Pittsburgh, Pittsburgh, Pennsylvania, United States of America Keywords: Biventricular repair-Mustard/Senning/Rastelli; ventricular septal defect; congenitally corrected transposition Correspsondence to: Marshall L. Jacobs MD, Section of Cardiothoracic Surgery, St. Christopher’s Hospital for Children, Erie Avenue at Front Street, Philadelphia, PA 19134, United States of America. Tel: +215 427 5109; Fax: +215 427 3860; E-mail: Marshall.Jacobs@tenethealth.com EXTRACARDIAC CONDUIT INTRAVENTRICULAR TUNNEL LV Ao PA CS PA RV Figure 1. Cartoon showing the essence of anatomic correction incorporating cre- ation of an atrial baffle combined with the Rastelli procedure. Discordant Atrioventricular Connections