Outcomes and risk factors for heart transplantation in children with end-stage cardiomyopathy†.

OBJECTIVES: Heart transplantation (HT) is the treatment of choice in children with end-stage cardiomyopathy. Several clinical, morphological, demographic, donor and recipient transplant factors have been demonstrated to affect survival in those patients following listing for HT and following HT. We aim to report our single institution results of HT in children with cardiomyopathy, and explore variables affecting survival and the need for heart retransplantation (RHT).

METHODS: Between 1988 and 2013, 125 children with cardiomyopathy underwent HT. Competing risks analysis modelled events after HT (RHT, death without RHT). Multivariable regression analysis examined risk factors affecting outcomes and parametric models were used to compare survival between diverse groups of patients.

RESULTS: There were 62 males (50%). Cardiomyopathy types were dilated (n = 104, 83%), restrictive (n = 10, 8%), chemotherapy-induced (n = 7, 6%), and other (n = 4, 3%). Median age at listing was 6.9 years and median age at HT was 7.0 years with median waiting list duration of 29 days. Thirty-four patients were infants <1 year. At time of HT, 106 patients (85%) were at United Network for Organ Sharing status-1, 25 (20%) were ventilated and 17 (14%) had mechanical circulatory support. There was 1 operative death. Competing risks analysis showed that at 10 years following HT, 10% of patients have undergone RHT, 32% have died without RHT and 58% of patients were alive without RHT. On multivariable analysis, risk factors for death following HT were panel-reactive antibodies >10% {hazard ratio [HR]: 4.1 [95% confidence interval (CI): 1.7-9.9], P = 0.002}, age group >10 years [HR: 3.2 (95% CI: 1.4-8.1), P = 0.009] and pre-HT mechanical circulatory support [HR: 2.9 (95% CI: 1.1-7.7), P = 0.033]. Additionally, earlier era <2000 was a significant risk factor for early phase mortality [HR: 8.7 (95% CI: 1.8-42.5), P = 0.017] but not for constant or late phase mortality [HR: 0.8 (95% CI 0.3-1.8), P = 0.6]. Following RHT, 6/11 (55%) expired yielding overall parametric survival estimates of 92, 77 and 57% at 1, 5 and 15 years, respectively.

CONCLUSIONS: Despite remarkable improvement in operative mortality and 1-year survival of children undergoing HT for cardiomyopathy in the current era, that advantage is reduced at the later follow-up, especially in teenagers indicating ongoing compliance and chronic management challenges. In children requiring pre-HT mechanical support, mid-term attrition is higher despite low operative mortality.