Long-term results after Fontan procedure and its modifications.

The Fontan procedure is an effective method of treatment for patients with tricuspid atresia, univentricular heart, and other complex lesions. Modifications of the Fontan procedure have been developed to treat various anatomic malformations. From 1975 to 1984, 31 patients (17 male and 14 female) have undergone 35 Fontan operations. The youngest patient was 4 and the oldest 26 years of age (average 12.2 years). Sixteen patients had tricuspid atresia, and 15 univentricular heart. Twenty-three patients had undergone 37 palliative operations before the Fontan procedure. The original Fontan procedure was performed in 10 patients until 1977, and there were three early deaths (30%). After that time, the operative technique was modified and the early mortality dropped to 12% (3/25 patients). A valved prosthetic conduit was used in 11 patients (right atrium to pulmonary artery in five; right atrium to right ventricle in six), a valveless conduit in 14 patients (right atrium to pulmonary artery in nine; right atrium to right ventricle in five), and direct anastomosis (right atrium to pulmonary artery) in 10 patients. Postoperative cardiac catheterization was performed in 20 patients and revealed excellent results in 15. The remaining five had reduced oxygen saturation; three of them had had a Glenn anastomosis 8 and 10 years before the Fontan procedure, and one had an anomaly of the coronary sinus with a shunt to the left atrium. This patient required reoperation. Three other patients underwent reoperation because of calcified stenotic and/or outgrown conduits. Postoperative mean right atrial pressure varied from 10 to 20 mm Hg. The hemodynamic ventricular parameters were within the normal range. We conclude that in patients with tricuspid atresia, univentricular heart, and other complex cardiac lesions, inactivating the right ventricular pump function by means of the Fontan procedure does not adversely affect the systemic ventricle. Although the response to exercise was abnormal, the clinical condition of these patients was good to excellent.