儿童心脏移植41例回顾性分析及随访研究

Retrospective reviews and follow-ups of 41 children after heart transplantation

目的:探讨儿童心脏移植风险因素及随访生存分析研究。方法:回顾性分析广东省人民医院2018年1月至2022年6月期间年龄<18岁的41例儿童心脏移植受者的围手术期资料以及3岁以下婴幼儿的供、受者相关资料。围手术期存活受者通过门诊及电话方式随访至2022年8月31日,观察指标包括随访期间生存情况、EB病毒(Epstein-Barr virus,EBV)感染情况等。使用Kaplan-Meire法统计术后受者生存情况,使用Logistic单因素回归模型分析影响围手术期生存结果的可能危险因素。结果:41例儿童受者术前年龄120个月(58~138个月),其中男性22例(53.66%)。原发病包括:心肌病37例,心脏恶性肿瘤2例及1例移植后排斥反应再次移植、1例丹农病(Danon)病合并心肌病变。8例受者术前行ECMO辅助治疗并成功过渡至心脏移植。2例接受ABO血型不相容型(ABO incompatible,ABOi)心脏移植,6例3岁以下婴幼儿供、受者体重比为(2.95±2.36)。围手术期17例发生一种或一种以上并发症,治疗包括连续性肾脏替代治疗(continuous renal replacement therapy,CRRT)9例(21.95%),气管切开3例(7.32%),延迟关胸或二次开胸探查6例(14.63%),急性移植物失功4例(9.76%)。5例围手术期死亡。导致围手术期死亡的主要危险因素包括术前ECMO辅助[风险比(HR):32.00,95%置信区间(CI):(2.83~361.79),P<0.05]、术前CRRT[HR:11.33,95%CI(1.15~111.69),P<0.05]及总胆红素升高[HR:1.02,95%CI(1.002~1.040),P<0.05]。随访结果:1例受者发生EBV感染相关性移植后淋巴增殖性疾病导致死亡,1例发生EBV相关性肝平滑肌瘤接受肝动脉栓塞治疗。41例受者累积存活率为85.37%。其中未使用ECMO组受者累积存活率为96.97%(P<0.05)。术前使用ECMO的儿童受者术后死亡率比未使用ECMO受者高(P=0.0013),但对围手术期存活受者进行随访,提示术前使用ECMO并不会影响受者随访期间存活(P=0.53)。ABOi组受者及3岁以下婴幼儿受者术后及随访期间均无死亡。结论:采用ABOi心脏移植及大体重供心心脏移植不影响婴幼儿受者围手术期及术后存活,术前ECMO辅助、总胆红素升高及术前使用CRRT是影响儿童心脏移植受者围手术期存活的危险因素。

Objective To explore the risk factors and follow-up outcomes of pediatric heart transplantation(HT).Methods Between January 2018 and June 2022,perioperative data are retrospectively reviewed for 41 pediatric HT recipients aged<18 years and donor-recipient weight data for infants aged under 3 years at Guangdong Provincial People's Hospital.Perioperative survivors are followed up until August 31,2022 through out patient visits and telephone calls.Postoperative survivals are examined by Kaplan-Meier method and possible risk factors for perioperative survival identify with Logistic regression.Results There are 22 boys and 19 girls with a median age of 120(58~138)months.After preoperative adjuvant therapy of extracorporeal membrane oxygenation(ECMO),8 cases had a successful transition to HT and 2 children underwent ABO incompatible(ABOi)HT.Six children aged under 3 years had a donor-recipient weight ratio of 2.95.Among 17 children,there are one or more complications,including continuous renal replacement therapy(CRRT,9 cases,21.95%),tracheotomy(3 cases,7.32%),delayed chest closure or redo of sternotomy(6 cases,14.63%)and acute graft dysfunction(4 cases,9.76%).Five children died during perioperative period.The possible risk factors for perioperative mortality include preoperative ECMO assistance[HR:32.00,95%CI:(2.83~361.79),P<0.05],preoperative CRRT[HR:11.33,95%CI:(1.15~111.69),P<0.05]and total bilirubin[HR:1.02,95%CI:(1.002~1.040),P<0.05].During follow-ups,one child died from Epstein-Barr virus(EBV)associated post-transplant lymphoproliferative disease;another case of EBV-associated hepatic leiomyoma underwent transcatheter arterial embolization.With an overall survival rate of 85.37%,the cumulative survival rate is 96.97%for children without preoperative ECMO assistance(P<0.05).Postoperative mortality rate spiked markedly in children with preoperative ECMO assistance(P=0.0013).However,follow-up results of perioperatively survivors indicate that preoperative usage of ECMO will not affect follow-up survival(P=0.53).In ABOi group or infants aged under 3 years,no mortality occurres postoperatively or during follow-ups.Conclusions In infant aged under 3 years,the strategies of ABOi HT and large-weight donor HT are both safe and effective and it has no effect upon perioperative and follow-up survivals.Preoperative ECMO assistance,total bilirubin and preoperative use of CRRT are risk factors for perioperative survival.