702–4 The Fontan Procedure for Pulmonary Atresia and Intact Ventricular Septum (PA and IVS): Operative and Late Results

Abstract

From 1973 through January 1, 1994, 860 Fontan procedures had been performed at the Mayo Clinic with 39 (4.5%) having been done on patients with PA and IVS. Age at surgery ranged from 1–10/12 to 21 years (median = 6). Thirty-eight of the 39 patients had had a total of 71 prior palliative cardiovascular procedures. Tricuspid valve (TV) and right ventricular (RV) size ranged from “minuscule” to a maximum of 55% of expected. Only four patients had significant right ventricular to coronary artery fistulae and only one of these was “right ventricular coronary dependent”. There were three operative deaths (7.7%) and have been two sudden unexpected late deaths (2-1/2, and 8 years post Fontan). One patient had a successful cardiac transplant seven years post Fontan. Present ages of survivors ranged from 3 to 29 years (median = 11.5 years) with a median post Fontan follow-up of 5.3 years. At recent follow-up, 33 of 34 survivors were in good or excellent condition and one was fair. We feel patients with PA and IVS are not candidates for a conventional two ventricle repair unless the TV and RV are at least 70% of expected size. In patients with dimensions between 50% and 70%, a two ventricle repair combined with a bidirectional cavopulmonary anastomosis may be feasible. In patients with TV and RV size less than 50% of expected, the Fontan procedure provides “definitive palliation” and early and late results of this approach are encouraging.