Outcomes of heart transplantation in children with hypoplastic left heart syndrome previously palliated with the Norwood procedure

Background Following the Norwood operation, unfavorable hemodynamic or anatomic factors might disqualify children from progressing through subsequent palliative surgeries necessitating listing for heart transplantation. Those patients often have immune, clinical, or anatomic risk factors that could preclude donor allocation, increase operative risk, and diminish late survival. We studied transplantation outcomes in those patients using the Pediatric Heart Transplant Study database. Methods A total of 253 children who had prior Norwood were listed for transplantation (1993-2012). Competing risks analysis modeled events after listing (death, transplantation) and after transplantation (death, retransplantation) and examined factors affecting outcomes. Results Patients were listed following Norwood (n = 89, 35%), Glenn (n = 96, 38%), and Fontan (n = 68, 27%). Competing risk analysis showed that 1 year after listing, 23% of patients had died, 70% had received transplantation, and 7% were alive without transplantation. Factors associated with death without transplantation included UNOS status I (HR 3.44 [1.58-7.49], P =.002) and mechanical circulatory support (HR 4.13 [2.04-8.34], P <.001). Overall, 188 patients received transplantation. Competing risk analysis showed that 10 years following transplantation, 40% had died, 7% had received retransplantation, and 53% were alive without retransplantation. Factors associated with death following transplantation were race other than white (HR 2.18 [1.19-3.99], P =.01), and donor mode of death other than anoxic brain injury (HR 2.23 [1.00-5.01], P =.05). Conclusions Heart transplantation can salvage children failing palliation following Norwood with outcomes comparable to those reported for other congenital cardiac anomalies. Efforts to stabilize patients and increase donor pool could improve survival by decreasing the high waiting list mortality. In patients who received transplantation, survival is not affected by last palliation stage or sensitization, reflecting current advances in perioperative management and immunosuppression management strategies.