原位心脏移植治疗婴幼儿心肌致密化不全1例并文献复习  

Treatment with orthotopic heart transplantation for infants with noncompaction of ventricular myo-cardium:one case report with a literature review

作　　者：莫绪明[1] 陈鑫[2] 彭卫[1] 蒋英硕[2] 陈涌[1] 陈润森 武开宏[1] 束亚琴[1] 陈凤 张玉喜[1] Mo Xuming;Chen Xin;Peng Wei;Jiang Yingshuo;Chen Yong;Chen Runsen;Wu Kaihong;Shu Yaqin;Chen Feng;Zhang Yuxi(Department of Thoracic&Cardiovascular Surgery,Affiliated Children's Hospital,Nanjing Medical University,Nanjing 210008,China;Department of Cardiovascular Surgery,Affiliated Hospital,Nanjing Medical University,Nanjing 210006,China)

机构地区：[1]南京医科大学附属儿童医院心胸外科,南京210008 [2]南京医科大学附属南京医院心胸血管外科,南京210006

出　　处：《中华小儿外科杂志》2022年第7期635-640,共6页Chinese Journal of Pediatric Surgery

基　　金：国家自然科学基金(81970265);国家重点研究发展计划(2017YFC1308105)。

摘　　要：目的总结1例婴幼儿心肌致密化不全(noncompaction of ventricular myocardium, NVM)应用心脏移植术的治疗经验, 讨论心脏移植治疗婴幼儿NVM的应用价值。方法 2020年8月南京医科大学附属儿童医院心胸外科收治了1例终末期NVM的患儿, 采用双腔静脉法进行原位心脏移植术, 于2020年8月12日对其成功实施了心脏移植术。通过PubMed、Springer Link、Google Scolar、Embase、中国生物医学文献数据库(CBMdisc)、中国知网(CNKI)、万方和维普数据库检索, 总结对婴幼儿NVM行心脏移植术的诊疗经验。结果患儿既往有心脏手术史, 术前彩色多普勒超声心动图检查提示左心室NVM、左心室壁运动减弱、二尖瓣中-大量反流。既往多次内科保守治疗后效果较差。心脏供体和受体ABO血型均为A型, 供体与受体的体重比值为1.21。供心冷缺血时间为332 min。受体主动脉阻断时间为114 min, 体外循环时间为241 min, 后并行辅助时间为130 min。术后机械通气时间为41 h。术后1年内无严重并发症, 未出现免疫排斥反应。检索文献, 仅发现3篇单中心英文病例报道, 共3例NVM婴幼儿接受心脏移植术, 均预后良好, 其中最长的随访时间为3年。结论运用双腔静脉法行原位心脏移植术可以作为婴幼儿NVM终末期治疗的有效手段。Objective To summarize the experiences of orthotopic heart transplantation for noncompaction of ventricular myocardium(NVM)in infants.Methods In August 2020,an infant with end-stage NVM was admitted into Affiliated Children's Hospital.Bicaval orthotopic heart transplantation(BOHT)was successfully performed on August 12,2020.The diagnostic and therapeutic experiences of heart transplantation in NVM infants were summarized by searching the database of PubMed,SpringerLink,Google Scholar,Embase,Ovid,Chinese Biomedical Literature(CBMdisc),Chinese National Knowledge Infrastructure(CNKI),WanFang and VIP.Results The infant had a previous history of cardiac surgery.Preoperative color Doppler echocardiography hinted at left NVM,hypomotility of left ventricular wall and moderate-severe mitral regurgitation.Poor response remained after conservative measures.ABO blood type of donor and recipient were both type A and donor/recipient body mass ratio was 1.21.The cold ischemic time of donor heart was 332 min.The duration of recipient aortic cross clamp,cardiopulmonary bypass and posterior parallel circulation were 114,241 and 130 min respectively.Postoperative mechanical ventilation duration was 41 hours.No serious complication or immune rejection occurred at Year 1 postoperatively.Three English case reports of NVM were retrieved.All of them underwent heart transplantation with a decent prognosis.The longest follow-up period was 3 years.Conclusions BOHT is an effective treatment of end-stage NVM in infants.

关 键 词：心脏移植 心肌致密化不全 婴幼儿 

分 类 号：R726.5[医药卫生—儿科]