Long-term follow-up of Fontan completion in adults and adolescents

The Fontan procedure is rarely performed in adults and adolescents in the present era. We review our results with the Fontan procedure in adolescents and young adults.

Between 1974 and 2010, 79 consecutive patients underwent the Fontan procedure at an age ≥ 15 years (mean age at Fontan operation, 20.3 years ± 4.5 years). Forty-five patients underwent atriopulmonary connection, 11 underwent the Bjork procedure, and 23 underwent total cavopulmonary connection.

Ten hospital deaths (HDs) and/or early Fontan takedowns (TDs) occurred. The median follow-up period was 18.2 years (range, 0.6-37.6 years). The estimated freedom from death or TD rates was 79.7% at 5 years, 77.0% at 10 years, 73.9% at 15 years, and 63.9% at 20 years. Age was not a predictor of HD and/or TD. Freedom from death or TD after 1998 was 69.1% at 5 years, 69.1% at 10 years, and 69.1% at 15 years, and before 1997 was 82.3% at 5 years, 79.0% at 10 years, 75.5% at 15 years, and 65.1% at 20 years; there were no significant differences between the two groups. In 19 late-death patients, nine (47.4%) experienced sudden death. Among these patients, five had known arrhythmias before sudden death.

In patients who were ≥15 years old, the surgical results of the Fontan operation were acceptable. Approximately half of the late deaths were sudden deaths, mainly occurring 10-20 years postoperatively.