RESULTS: There were 76 male patients (61%); median age was 3.8 years (interquartile range, 0.6-11.5). Sixteen patients (13%) underwent primary
heart transplantation, and 108 patients (87%) underwent prior surgical repair/palliation. Thirty-eight patients (31%) had 2 ventricles, and 86 patients (69%) had a single ventricle, including 33 (27%) who underwent a prior
Fontan procedure. Competing risks analysis showed that
at 10 years after
heart transplantation, 13% of patients had undergone retransplantation, 43% of patients had died without retransplantation, and 44% of patients were alive without retransplantation. After retransplantation, 9 of 17 patients were alive and 3 of 17 patients had undergone a second retransplantation. Overall 15-year survival after initial
heart transplantation was 41%. On multivariable analysis, risk factors for early-phase mortality were age less than 1 year (hazard ratio [HR], 7.2; 95% confidence interval [CI], 2.4-22.0; P < .001) and prolonged
cardiopulmonary bypass (HR, 5.0; 95% CI, 2.1-11.8; P < .001). Risk factors for late-phase mortality were age more than 1 year (HR, 3.0; 95% CI, 1.1-7.7; P = .025) and donor-recipient race mismatch (HR, 2.2; 95% CI, 1.2-4.1; P = .016). Survival was not affected by era, underlying anomaly, prior
Fontan procedure, sensitization, or pulmonary artery augmentation.
CONCLUSIONS: The outcomes of
heart transplantation in children with
congenital heart disease have not improved in the current era. Survival was not affected by the underlying anomaly, prior
Fontan procedure, or sensitization. Strategies to improve the outcomes in patients with
congenital heart disease may need to address selection criteria,
transplantation timing, and pretransplant and post-transplant care. The effect of donor-recipient race mismatch warrants further investigation and might affect organ allocation algorithms or immunosuppression management.