Lessons learned from 119 consecutive cardiac transplants for pediatric and congenital heart disease

Background This manuscript reviews all patients who underwent orthotopic heart transplantations (OHT) at our program (116 patients underwent 119 OHT) to describe their diagnostic characteristics and to assess risk factors for mortality. Methods Median age at OHT was 179 days (mean, 1,446.6 ± 188.9 days [4.0 ± 0.5 years]; range, 5 days to 7,125 days [19.5 years]; 15 neonates, 68 infants). Median weight at OHT was 5.5 kg (mean, 17.2 ± 2.1 kg; range, 2.2 to 113 kg). Diagnoses were cardiomyopathy (n = 37), primary transplantation for hypoplastic left heart syndrome (HLHS) or HLHS-related malformation (n = 29), transplantation after prior cardiac surgery for HLHS or HLHS-related malformation (n = 9), non-HLHS congenital heart disease (n = 39), and retransplant (n = 5). Results Overall Kaplan-Meier 5-year survival was 72.7%. Operative mortality was 12.6% (15 patients). Late mortality was 13.4% (16 patients). Eighty-five patients survived, with a mean follow-up of 5.76 ± 0.48 years (median, 5.1 years; range, 0.12 to 14.0 years). Total follow-up was 507.0 years. No survival difference was seen among the five diagnostic subgroups (p = 0.20). Univariate association between risk factors and survival was assessed for the following variables: age (p = 0.91), weight (p = 0.86), sex (p = 0.47), race (p = 0.40), insurance classification (p = 0.42), high PRA (p = 0.20), pretransplant mechanical circulatory support (p < 0.001), posttransplant mechanical circulatory support (p < 0.001), redo sternotomy (p = 0.07), heterotaxy (p = 0.02), cardiopulmonary bypass time (p = 0.01), and donor heart cross-clamp time (p = 0.02). Conclusions Excellent results are expected for children undergoing OHT regardless of diagnostic classification. Pretransplant mechanical circulatory support, posttransplant mechanical circulatory support, cardiopulmonary bypass time, donor heart cross-clamp time, and heterotaxy are risk factors for decreased survival.