Jacobs Jeffrey P, Quintessenza James A, Boucek Robert J, Morell Victor O, Botero Luis M, Badhwar Vinay, van Gelder Hugh M, Asante-Korang Alfred, McCormack Jorge, Daicoff George R
The Congenital Heart Institute of Florida, All Children's Hospital, University of South Florida, St. Petersburg, Florida, USA.
Ann Thorac Surg. 2004 Nov;78(5):1703-9. doi: 10.1016/j.athoracsur.2004.03.031.
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%).
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.
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).
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.
高群体反应性抗体(PRA)可能被视为小儿原位心脏移植的一个风险因素。我们回顾性分析了高PRA(>10%)患儿心脏移植的管理策略和结果数据。
连续60例患儿(中位年龄=130.5天)接受心脏移植。诊断包括左心发育不全综合征(HLHS)(30例)、心肌病(18例)和术后复杂先天性心脏病(CCHD)(12例)。标准诱导免疫抑制治疗包括静脉注射类固醇、γ球蛋白和多克隆兔抗胸腺细胞球蛋白。初始免疫抑制药物为钙调神经磷酸酶抑制剂和抗增殖药物。8例患儿PRA升高(P组)。52例患儿PRA未升高(N组)。P组免疫抑制调整如下:术前静脉注射免疫球蛋白G(IVIG)和/或环磷酰胺或霉酚酸酯,术前和术后进行换血或血浆置换。P组中,环磷酰胺是初始抗增殖药物。
P组=4例HLHS患儿(均为心脏手术术后)和4例术后CCHD患儿。N组=26例HLHS患儿(4例心脏手术术后)、18例心肌病患儿和8例术后CCHD患儿。P组患儿年龄更大、体重更重。两组供心等待时间、心脏缺血时间和住院时间相似。P组30天死亡率为25%,N组为7.9%(p=0.178)。P组总死亡率为50%,N组为15.4%(p=0.043)。
尽管心脏移植可为终末期心力衰竭且PRA升高的患儿提供唯一的生存机会,但尽管进行了积极的免疫抑制,这些患者仍属于高风险。