Outcomes and risk factors for heart transplantation in children with congenital heart disease

Objective: Heart transplantation in children with underlying congenital heart disease has traditionally been associated with inferior outcomes. We report our single institution experience. Methods: Between 1988 and 2013, 124 children with congenital heart disease underwent heart transplantation. Competing risks analysis modeled events after heart transplantation (retransplantation, death without retransplantation). Multivariable regression analysis examined risk factors affecting outcomes. 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 posttransplant care. The effect of donor-recipient race mismatch warrants further investigation and might affect organ allocation algorithms or immunosuppression management.