特发性巨细胞性心肌炎行心脏移植术12例受者病例特点及预后研究  

Case characteristics and outcomes of idiopathic giant cell myocarditis after heart transplantation

作　　者：胡小莹 廖中凯 孙慧 黄洁 刘盛 王巍[2] 宋云虎[2] 胡盛寿[2] 王红月[3] 郑哲[2] Hu Xiaoying;Liao Zhongkai;Sun Hui;Huang Jie;Liu Sheng;Wang Wei;Song Yunhu;Hu Shengshou;Wang Hongyue;Zheng Zhe(Department of Cardiology,Fuwai Hospital,Chinese Academy of Medical Sciences,National Center for Cardiovascular Diseases,Beijing 100037,China;Department of Cardiac surgery,Fuwai Hospital,Chinese Academy of Medical Sciences,National Center for Cardiovascular Diseases,Beijing 100037,China;Pathology Department,Fuwai Hospital,Chinese Academy of Medical Sciences,National Center for Cardiovascular Diseases,Beijing 100037,China)

机构地区：[1]国家心血管病中心、中国医学科学院阜外医院心内科,北京100037 [2]国家心血管病中心中国医学科学院阜外医院心外科,北京100037 [3]国家心血管病中心、中国医学科学院阜外医院病理科,北京100037

出　　处：《中华器官移植杂志》2022年第12期718-722,共5页Chinese Journal of Organ Transplantation

摘　　要：目的:分析12例特发性巨细胞性心肌炎(idiopathic giant cell myocarditis,IGCM)行心脏移植术受者的病例特点及预后。方法:回顾性分析了2004年6月至2022年5月在中国医学科学院阜外医院行心脏移植后病理证实为IGCM的12例受者的临床资料。分析归纳受者的一般情况、临床表现、组织病理学检查结果、术后随访等情况。结果:2004年6月至2022年5月在中国医学科学院阜外医院进行心脏移植共1143例,术后组织病理学检查证实为IGCM12例,占1.05%。IGCM受者年龄(47.6±7.3)岁,男性5例(41.7%)。12例IGCM受者中首发临床表现为充血性心力衰竭7例(58.3%);心律失常4例(33.3%);心肌缺血1例(8.3%)。IGCM受者左室射血分数32.8%±11.1%,11例(91.7%)左室射血分数<50%,9例(75.0%)合并心律失常。从症状发作到心脏移植中位时间6个月。1例受者为首次因IGCM于外院行心脏移植,术后自行停用糖皮质激素及免疫抑制剂,11年后IGCM复发再次行心脏移植治疗。所有受者术前均未明确诊断,术前诊断为扩张型心肌病5例(41.7%)、致心律失常性右室心肌病3例(25%)。12例IGCM受者心脏移植术后随访4~142个月,1例于围术期死亡、1例于术后68个月因心力衰竭死亡,受者术后均规律服用激素及免疫抑制剂,仅有1例移植后9年出现1R级移植心脏排斥反应,无IGCM复发病例。经Kaplan-Meier生存分析,IGCM受者心脏移植术后1年、5年、10年的累积存活率分别为91.7%、91.7%及73.3%,与其他病因心脏移植受者比较,存活率差异无统计学意义(Log Rank P=0.265)。结论:心脏移植是治疗终末期IGCM的有效手段,术后规律足量的免疫抑制治疗对预防移植心脏排斥反应及IGCM复发至关重要,IGCM受者行心脏移植术预后良好。Objective To analyze the case characteristics and outcomes of 12 idiopathic giant cell myocarditis(IGCM)cases after heart transplantation(HT).Methods From June 2004 to May 2022,clinical data were retrospectively reviewed for 12 cases with pathologically confirm IGCM after HT at Fuwai Hospital.General characteristics,clinical manifestations,pathological examinations and postoperative follow-ups are recorded.Results From June 2004 to May 2022,a total of 1143 HT operations are performed at Fuwai Hospital and 12 cases of IGCM(1.05%)are confirmed by postoperative pathology.The age is(47.6±7.3)years.There are 5 boys and 7 girls.Initial presenting manifestations are congestive heart failure(7 cases,58.3%)and arrhythmia(4 cases,33.3%).Median time from symptom onset to HT is 6 months.All of them are undiagnosed pre-operation.And dilated cardiomyopathy(5 cases,41.7%)and arrhythmogenic right ventricular cardiomyopathy(3 cases,25%)are confirmed.The follow-up period is(4~142)months post-HT.One death occurred during perioperative period and another is due to heart failure at 68 months post-HT.Only 1 case of grade 1R transplant heart rejection occurrs at 9 years post-HT and there is no case of recurrence.According to Kaplan-Meier survival analysis,cumulative survival rates of 1/5/10 years post-HT in IGCM patients are 91.7%,91.7%and 73.3%respectively.No significant difference exist in survival rate for other etiologies post-HT(Log-rank P=0.265).Conclusions HT is efficacious for end-stage IGCM.Regular and sufficient postoperative immunosuppression is vital for preventing heart transplant rejection and recurrent IGCM.Most IGCM patients have a decent prognosis post-HT.

关 键 词：心脏移植 特发性巨细胞性心肌炎 预后 

分 类 号：R654.2[医药卫生—外科学]