THE FONTAN PROCEDURE FOR PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM - OPERATIVE AND LATE RESULTS

Objectives. The goals of the study were to evaluate the operative and late mortality associated with the Fontan procedure in patients with p ulmonary atresia and an intact ventricular septum and to obtain follow -up information on the current clinical status of surviving patients. Background. Between 1979 and October 1, 1995, 40 patients with the ano maly had a nonfenestrated Fontan procedure performed at the Mayo Clini c. Because there are no previously published reports involving a serie s of this size in which the Fontan approach was used for this conditio n, a review of patient outcomes was thought to be of value. Methods. T he medical records of the 40 patients were reviewed retrospectively, a nd 34 were determined to be alive. The status of the survivors as of l ate 1995 was then ascertained by direct examination, questionnaire or telephone follow-up. Results. There were three operative deaths and th ree late deaths. The current ages of the 34 survivors ranged from 4 to 30 years (median 13). Thirty-three of the 34 survivors were thought t o be in New York Heart Association functional class I or II, and all b ut three of these patients, of school age or older, were either full-t ime students or working full time. The three adults who were not emplo yed thought they were capable of working but were not doing so because of socioeconomic reasons. More than half of the patients were not rec eiving cardiovascular medications, Conclusions. These overall gratifyi ng early and late results encourage continued application of this oper ation for appropriately selected patients with this complex congenital cardiovascular anomaly. (C) 1997 by the American College of Cardiolog y.