Fontan Completion in Infants

Background Since the implementation of the Fontan procedure, several clinical factors have been linked to outcome. A study of the outcome after Fontan completion was undertaken with particular attention to the influence of age and cardiac diagnosis. Methods Review of all patients (n = 107) undergoing Fontan completion between January 1998 and July 2005 to identify predictors of outcome: early death, prolonged effusions, and prolonged hospital stay. Results Median age was 13 months (range, 11 to 35) and median weight was 9.4 kgs (6.7 to 15.1). Hypoplastic left heart syndrome was present in 61 patients, and stage I Norwood was the initial palliation in 69. An interim superior cavopulmonary connection was performed in all. A lateral tunnel was used, and a deliberate right to left shunt was created in 99 patients. Mean transpulmonary gradient and pulmonary artery pressure were 5.7 ± 1.5 mm Hg and 11.6 ± 2.2 mm Hg, respectively. Median time to extubation was 5 hours (range, 2.5 to 184). Median duration of pleural effusion was 3 days (1 to 58) and was greater or equal to 14 days in 13 patients (12%). Overall mortality was 4.5% (5 of 107). Variables associated with poor outcome included associated noncardiac diagnosis (p< 0.05), elevated transpulmonary gradient (p = 0.03), and pulmonary artery pressure (p< 0.02). Hypoplastic left heart syndrome was the only variable associated with prolonged effusive complications. Conclusions Fontan completion can be performed with good results in the first year of life independent of anatomic diagnosis. Significant noncardiac pathology, and a higher pulmonary artery pressure were predictive of worse outcome. Hypoplastic left heart syndrome was associated with prolonged effusions.