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Heart Transplantation in Children after a Fontan Procedure: Better than People Think
1Division of Cardio-Thoracic Surgery, Department of Surgery, Emory University School of Medicine, Atlanta, GA.
Insights
Pediatric heart transplantation outcomes are similar for children with a prior Fontan procedure compared to those without. Careful selection ensures comparable survival rates and rejection incidence in Fontan patients post-transplant.
Area of Science:
- Cardiology
- Pediatric Surgery
- Transplantation Medicine
Background:
- The Fontan procedure is a palliative surgery for complex congenital heart defects.
- Previous studies suggested poorer outcomes for heart transplantation following a Fontan procedure.
- The long-term impact of Fontan circulation on heart transplant success requires further investigation.
Purpose of the Study:
- To evaluate survival rates and clinical outcomes of pediatric heart transplantation in patients with a prior Fontan procedure.
- To compare outcomes between children undergoing primary heart transplantation with and without a history of the Fontan procedure.
- To identify factors influencing transplant success in this specific patient population.
Main Methods:
- Retrospective analysis of 273 primary pediatric heart transplants performed since 1988.
- Comparison of clinical characteristics, peri-operative data, and post-transplant outcomes between Fontan and non-Fontan patient groups.
- Statistical analysis of survival, retransplantation rates, and rejection incidence.
Main Results:
- Fontan patients were older at transplant and had more complex surgical histories, including higher rates of pulmonary artery reconstruction.
- Despite longer ischemic and bypass times, 1-year and 5-year actuarial survival rates were similar between Fontan and non-Fontan groups.
- Retransplantation rates were higher in the Fontan group, but early and mid-term survival showed no significant disadvantage.
Conclusions:
- Children undergoing heart transplantation after a Fontan procedure do not experience an early or mid-term survival disadvantage.
- Carefully selected pediatric patients with failing Fontan circulation can achieve comparable outcomes to other heart transplant recipients.
- The findings challenge previous reports and support heart transplantation as a viable option for select Fontan patients.
Abstract:
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.

