Heart Transplantation in Children after a Fontan Procedure: Better than People Think.

Previous studies have reported that children with a prior Fontan procedure have decreased survival after heart transplantation. We examined 273 primary pediatric heart transplants. Since 1988, 33 (12.1%) of 273 children <18 years old undergoing primary heart transplantation had a Fontan procedure 3.7 ± 4.3 years before transplantation. Compared with 240 (87.9%) non-Fontan primary transplants, the Fontan patients were older (8.8 ± 5.2 vs 6.6 ± 5.9 years; P = .023), but were similar in presensitization and pre-transplant clinical status. More Fontan patients had prior operations (100% vs 51.7%; P < .0001) and needed pulmonary artery reconstruction (100% vs 21.7%; P < .0001). Thirteen (39%) had protein-losing enteropathy. Donor ischemic times (213 ± 73 vs 177 ± 57 minutes; P = .0013) and cardiopulmonary bypass times (199 ± 86 vs 125 ± 53 minutes; P < .0001) were greater in the Fontan group, as were durations of ventilator support (4.4 ± 6.0 vs 2.5 ± 4.3 days; P = .035) and hospital stay (18.6 ± 16.1 vs 14.7 ± 13.1 days; P = NS). The Fontan group had one 30-day mortality. One-year actuarial survival (84.8% vs 86.9%, Fontan vs non-Fontan) and 5-year actuarial survival (70.8% vs 70.3%, Fontan vs non-Fontan) were similar, as was rejection incidence at 1 year (2.0 ± 2.0 vs 1.7 ± 1.9 episodes/patient; P = .3972). Five Fontan patients (18.5%) required retransplantation 4.9 ± 3.6 years post-transplant, compared with 22 non-Fontan patients (9.2%) retransplanted 5.2 ± 3.4 years post-transplant. Contrary to prior reports, we did not identify any early or mid-term disadvantage for children undergoing heart transplantation after a previous Fontan procedure, despite more complex transplant operations. We contend that carefully selected children with a failing Fontan circulation can do as well as other children with heart transplantation.