Outcomes of Patients Undergoing Primary Fontan Operation Beyond First Decade of Life

Studies on older patients undergoing primary Fontan operation (FO) are limited, with conflicting results. We review our experience with these patients beyond the first decade of life.

Between January 2000 and December 2014, a total of 105 patients ≥10 years of age (mean 15.6 ± 4.9, range 10-31, median 15 years) underwent primary FO without a prior bidirectional superior cavopulmonary anastomosis (Bidirectional Glenn [BDG]). Mean preoperative New York Heart Association (NYHA) class was 2.2 ± 0.57.

Operative procedure was extra-cardiac FO in 62 patients (8 were fenestrated). Forty-three had a lateral tunnel FO (26 were fenestrated). There were 11 (10.5%) early deaths. Fourteen of the 94 early survivors experienced prolonged pleural effusions, 7 had arrhythmias, and 2 had thromboembolic events. Two patients underwent Fontan takedown. On univariate analysis, NYHA functional class III, mean pulmonary artery (PA) pressure ≥15 mm Hg, hematocrit ≥60%, preoperative ventricular dysfunction, and atrioventricular valve regurgitation (AVVR) were associated with early mortality. Median follow-up was 78 (mean 88.9 ± 6.3) months. In 94 survivors, 6 (6.4%) late deaths were encountered. At last follow-up, 81 (86.2%) survivors were in NYHA class I. Actuarial survival was 84.7% ± 3.7% at 5, 10, and 15 years.

Carefully selected adolescents and young adults can safely undergo the primary FO. However, persistent pleural effusions, arrhythmias, thromboembolic events, and the need for reoperation mandate regular follow-up in such patients. Preoperative NYHA functional class III, mean PA pressure ≧ 15 mm Hg, hematocrit ≥ 60%, ventricular dysfunction, and AVVR were associated with early mortality, suggesting that primary FO should be avoided in such patients.