Somatic development long after the Fontan operation: Factors influencing catch-up growth

Objective: As mortality and morbidity after the Fontan operation has improved, long-term outcome, including developmental aspects, have become more important. To understand the long-term effects of this operation, we followed somatic development for up to 15 years. Methods: We evaluated 90 patients who underwent the Fontan operation between 1984 and 2004 (mean follow-up, 11.8 ± 4.2 years). The modified Fontan operations were atriopulmonary anastomosis (n = 19) and total cavopulmonary connection (n = 71). Mean age at the time of surgical intervention was 5.5 ± 4.8 years. Weight, height, and body mass index were evaluated preoperatively and postoperatively and given as percentiles on a normal growth curve. Results: Postoperative weight, height, and body mass index reached the 47.2 ± 35.6, 37.9 ± 30.4, and 41.6 ± 31.2 percentiles, which were significantly better than preoperative values (the 21.6 ± 25.9, 25.9 ± 25.7, and 20.0 ± 25.1 percentiles). Although neither early surgical intervention nor anatomic features affected postoperative growth, early Fontan completion demonstrated better somatic development in subgroups of tricuspid atresia. Prior bidirectional Glenn shunting provided better weight gain before the Fontan operation. Prior atrioseptectomy, central shunt, and pulmonary artery reconstruction were associated with impaired somatic development. Reoperation and catheter-based intervention improved somatic development. Conclusions: Long-term catch-up growth can be observed in patients after the Fontan operation. Early volume-unloading procedures might lead to better somatic growth. Prior atrioseptectomy, central shunt, and pulmonary artery reconstruction are associated with impaired weight and height gain, implying that the severity of the underlying diseases affects postoperative somatic development.