Tricuspid atresia is an uncommon form of
congenital heart disease and long-term survival was rare before the Fontan era. It was thought that the long-term survival of patients with
tricuspid atresia would be improved by the introduction of the
Fontan procedure and its subsequent modifications. This study reviews the
clinical course of 84 patients with
tricuspid atresia identified in the first year of life in the Fontan era. Prior palliative operations, their results and their ultimate application for the
Fontan procedure were considered. Eleven patients died before surgical intervention and 5 did not undergo catheterization or echocardiographic confirmation before death. Five children underwent the
Fontan procedure without prior palliation and 1 child does not require palliation at the present time. Sixty-seven patients (80%) had
surgical procedures before evaluation for the suitability of a
Fontan operation. Thirty-four patients had a second surgical palliation and 9 patients had a third palliation. The surgical mortalities for the first, second and third
palliative surgery were 17.9, 17.6 and 0%, respectively. Thirty-two patients (38%) underwent the
Fontan procedure and 2 deaths occurred (6%). An estimate of the probability of surviving for 1 year was 64% (95% confidence limits 54 to 74%) and that of 8 years was 55% (95% confidence limits 44 to 66%).