Heart Transplantation in Adult Congenital Patients Performed at a Pediatric Center: An Institutional Experience

Abstract

<h3>Purpose</h3> Advancements in pediatric cardiovascular surgery have improved outcomes of children resulting in more adults with palliated congenital heart disease requiring a heart transplant. There is no standardized practice among adult congenital transplant centers for the location of pre- and posttransplant management of these patients. <h3>Methods</h3> We present the outcomes of 17 adult congenital transplantations performed at our pediatric institution from 2007-2021 as a joint evaluation and management effort between adult and pediatric programs, with pre, peri- and post-operative management occurring at the pediatric center. <h3>Results</h3> Patients aged 18-47 years at the time of heart transplantation. The most common indication for transplant was single ventricle with failing Fontan physiology, followed by DTGV with failing Mustard physiology. Two patients were retransplants for coronary artery vasculopathy. Patients listed were deemed candidates by both the pediatric and adult transplant teams. All patients were admitted prior to transplant at the pediatric hospital for initiation of inotropic therapy. The average pretransplant hospital stay was 76.9 days. Three patients required ECMO following transplant, for an average of 2 days. All patients received thymoglobulin immunosuppression and IV steroids. Maintenance immunosuppression was achieved with tacrolimus, mycophenolate mofetil, and oral steroid wean. Posttransplant length of stay averaged 15.8 days. All patients required temporary pacing. None required permanent pacing post-transplant. Most developed transient liver insufficiency and transient renal insufficiency. To date, 15/17 patients are living. One patient died of rejection at 22 months posttransplant and the other patient had massive post-transplant hemorrhage and developed mediastinitis, liver and renal failure and died 31 days posttransplant. The one year survival in this cohort was 94%. The three and five year survival is 88%. <h3>Conclusion</h3> We recommend a combined approach by adult and pediatric heart transplant teams when caring for adult congenital patients undergoing heart transplantation. Emphasis should be placed on the optimal location for congenital surgical management. Outcomes at or above the national adult heart transplant average can be achieved with such management in a high risk population.