Fontan's operation: an expanded horizon

The Fontan principle of redirecting systemic venous blood into the pulmonary arteries via a conduit has now gained a much wider application than initially intended. We have applied this type of operation in a series of 15 patients, five of whom had "classical" tricuspid atresia with concordant arterial connections. The other 10 patients showed a variety of congenital malformations, such as double-inlet univentricular heart with discordant arterial connections, double-outlet right ventricle with common atrioventricular canal. Ebstein's anomaly with almost imperforate tricuspid valve, hypoplasia of right ventricular inflow, and a crisscross heart with straddling right atrioventricular valve. Important associated anomalies, such as common atrium and anomalous pulmonary and/or systemic drainage, required particular adaptation of the technique. Thirteen of the 15 patients survived operation and are presently alive. One death occurred in a patient in whom the preoperative diagnosis had to be adjusted during operation because of unexpected total anomalous pulmonary venous connections. The other death occurred in a patient suffering from the "asplenic syndrome." The autopsy in this patient suggested that the large baffle used for atrial septation and ventricular exclusion had obstructed pulmonary venous return. Our experience thus supports the opinion that Fontan's operation has a place in the treatment of otherwise anatomically uncorrectable lesions.