Background: Elevated panel reactive antibody (PRA) may be considered a risk factor precluding pediatric orthotopic heart transplantation. We retrospectively reviewed our management strategy and outcome data for children undergoing heart transplantation with high PRA (> 10%).
Methods: Sixty consecutive children (median age = 130.5 days) underwent heart transplantation. Diagnoses included hypoplastic left heart syndrome (HLHS) (30 patients), cardiomyopathy (18 patients), and postoperative complex congenital heart disease (CCHD) (12 patients). Standard induction immunosuppressive therapy included pulse steroids, gamma globulin, and polyclonal rabbit antithymocyte globulin. Initial immunosuppression is a calcinurin inhibitor and an antiproliferative agent. Eight children exhibited elevated PRA (group P). Fifty-two exhibited nonelevated PRA (group N). Immunosuppression was modified in group P as follows: preoperative intravenous immunoglobulin G (IVIG) and/or cyclophosphamide or mycophenolate mofetil and preoperative and postoperative exchange transfusions or plasmapheresis. In group P, cyclophosphamide was the initial antiproliferative agent.
Results: Group P = 4 HLHS patients (all status post [s/p] prior cardiac surgery) and 4 postoperative CCHD patients. Group N = 26 HLHS patients (4 patients s/p prior cardiac surgery), 18 cardiomyopathy patients, and 8 postoperative CCHD patients. Group P patients were older and weighed more than group N patients. Waiting time for donor heart, cardiac ischemic time, and length of hospital stay were similar in both groups. Thirty-day mortality for group P was 25% and for group N it was 7.9% (p = 0.178). Overall mortality for group P was 50% and for group N it was 15.4% (p = 0.043).
Conclusions: Although heart transplantation can offer children with end-stage heart failure and elevated PRA their only chance of survival, these patients remain high risk despite aggressive immunosuppression.