Transplantation for Congenital Heart Disease in Children-A Quest to Do Better

Children with congenital heart disease (CHD) represent the largest group of pediatric heart transplant recipients but experience lower graft survival than those transplanted for cardiomyopathy. We evaluated post-transplant outcomes among CHD recipients at the Hospital for Sick Children (HSC) and identified predictors of graft loss. Pediatric patients (< 17 years) undergoing heart transplantation for CHD between 1993 and 2023 were analyzed using the Pediatric Heart Transplant Society registry. Outcomes of CHD recipients at HSC (CHD HSC) were compared with cardiomyopathy HSC recipients, CHD recipients at other centers (CHD Non-HSC), and a propensity score-matched (PSM) cohort adjusting for pre- and peri-transplant risk factors. Kaplan-Meier survival and multivariable Cox proportional hazards analyses were performed. Among 4,362 pediatric CHD transplant recipients, 152 underwent transplantation at HSC. Freedom from graft loss for CHD HSC at 5, 10, and 15 years was 71.8%, 63.0%, and 58.4%, lower than in Cardiomyopathy HSC recipients (p < 0.0001), but similar to CHD Non-HSC (p = 0.6043). Survival remained comparable in the PSM cohort (n = 140, p = 0.4599). Conditional survival beyond 1 year was similar between groups, suggesting early mortality drives differences. CHD HSC recipients demonstrated longer freedom from CAV (p = 0.0041) and infection (p = 0.0335). Predictors of graft loss included pre-transplant ECMO (HR 3.11), ICU admission (HR 2.39), PRA > 10% (HR 2.49), African American/Black race/ethnicity (HR 2.71), and longer waitlist duration (HR 1.06). Graft survival in CHD recipients at HSC remains lower than in cardiomyopathy recipients but comparable internationally. Optimizing pre-transplant care, sensitization management, and equity-focused interventions may enhance outcomes.