Said, Sameh M.

The Fontan procedure is now considered the final common pathway for patients with anatomical or functional single ventricle. These patients initially have their systemic and pulmonary circulations in parallel, supported by one functional ventricular chamber. The ultimate goal with this procedure is to separate the two circulations, to prevent mixing of venous and arterial blood, and to provide adequate tissue oxygenation. The objective of this article is to review the Fontan procedure with its various modifications and refinements since its introduction to clinical practice in 1971, by Fontan and Baudet.

Pulmonary artery translocation has been described as an alternative surgical strategy to treat anomalous aortic origin of the coronary artery from the wrong sinus of Valsalva. We describe another application of this strategy in a patient who underwent complete repair of pulmonary atresia with ventricular septal defect and presented years later with ischemic symptoms due to compression of the left main coronary artery by the right ventricular-to-pulmonary artery conduit. To our knowledge, this is the first application of the pulmonary artery translocation technique to treat such a problem.

In patients with congenitally corrected transposition of the great arteries, the main concern has been the long-term performance of the morphologic right ventricle in association with tricuspid valve regurgitation when it remains as the systemic ventricle. Deterioration in ventricular function can occur slowly over many years, even without associated cardiac anomalies or previous surgical interventions. This review summarizes the authors' experience and provides a thorough review of the literature addressing the management of the failing systemic right ventricle as well as the tricuspid valve regurgitation with congenitally corrected transposition. This includes different surgical options, the authors' preferred management algorithm, and the late outcome.