动脉调转联合主动脉弓部重建一期矫治Taussig-Bing畸形合并主动脉弓部梗阻性病变  

Single-stage arterial switch with aortic arch reconstruction for Taussig-Bing anomaly with aortic arch obstruction

作　　者：邹明晖[1] 马力[1] 夏园生[1] 杨盛春[1] 陈伟丹[1] 陈欣欣[1] Zou Minghui;Ma Li;Xia Yuansheng;Yang Shengchun;Chen Weidan;Chen Xinxin(Department of Cardiac Surgery,Guangzhou Women and Children's Medical Center,Guangzhou Medical University,Guangzhou 510623,China)

机构地区：[1]广州医科大学附属广州市妇女儿童医疗中心心脏中心,510623

出　　处：《中华胸心血管外科杂志》2018年第12期717-720,共4页Chinese Journal of Thoracic and Cardiovascular Surgery

摘　　要：目的评价动脉调转联合主动脉弓部重建一期矫治Taussig-Bing畸形或大动脉转位(TGA)合并主动脉弓部梗阻性病变的手术效果。方法回顾性分析2009年11月至2015年12月,26例诊断为TGA或Taussig-Bing畸形合并主动脉弓部梗阻,并接受一期动脉调转和主动脉弓部重建患儿的临床资料、手术资料和术后近中期结果。结果全组20例Taussig-Bing畸形,6例TGA。主动脉弓部梗阻包括:主动脉缩窄20例,主动脉弓离断6例。接受手术时年龄11~630天,中位值52天;体质量2.1~9.3 kg,中位值3.6 kg。手术早期死亡3例,占11.5%。随访24~103个月,平均(44±23)个月,随访期间死亡2例。1年和5年实际生存率分别为84%(95%CI:70%~98%)和77%(95%CI:58%~96%)。随访发现,新主动脉瓣轻度反流3例,主动脉瓣上狭窄2例,复发主动脉弓降部再缩窄1例;7例出现右心系统梗阻病变,肺动脉瓣轻中度反流2例。6例行再次手术干预,其中4例因右心系统梗阻再手术。总体1年和5年免于再手术比例分别为:90%(95%CI:78%~99%)和59%(95%CI:32%~86%),免于右心系统再手术比例分别为:94%(95%CI:84%~99%)和66%(95%CI:37%~95%)。无再手术死亡病例。结论一期动脉调转联合主动脉弓部重建治疗TGA或Taussig-Bing畸形合并主动脉弓部梗阻性病变,可取得满意的近中期生存率。尽管晚期右心梗阻性病变风险和再干预风险仍较高,我们并不支持初期手术时行跨环补片来扩大右心室流出道。最佳手术方式有待于进一步探讨。Objective To review the surgical outcomes of single-stage arterial switch with aortic arch reconstruction for Taussig-Bing anomaly with aortic arch obstruction in a single institution of china.Methods The clinical data of 26 consecutive patients with Taussig-Bing anomaly or TGA and aortic arch obstruction undergoing single-stage arterial switch with aortic arch reconstruction in Guangzhou Women and Children's Medical Center from November 2009 to December 2015 were retrospectively reviewed, and the risk factors for re-intervention were analyzed.Results There were three in-hospital deaths, and the early-term survival rate was 88.5%(23/26). Follow-up data were available for all patients who survived the operation(range 24-103 months). There were two late deaths. Survival estimates for the entire cohort following surgery were 84%(95%CI: 70%-98%) and 77%(95%CI: 58%-96%) at 1 year and 5 years, respectively. Echocardiology showed three cases of aortic regurgitation, two cases of supra-aortic stenosis, one case of recoarctation, seven cases of right heart outflow tract obstruction, and two cases of moderate pulmonary regurgitation. Six patients required a re-intervention during the follow-up period with no mortality. Freedom from re-intervention were 90%(95%CI: 78%-99%) and 59%(95%CI: 32%-86%) at 1 year and 5 years, respectively. Freedom from re-intervention for right-sided outflow tract obstruction were 94%(95%CI: 84%-99%) and 66%(95%CI: 37%-95%) at 1 year and 5 years, respectively. All survivors remained in good condition(New York Heart association functional class Ⅰ or Ⅱ).ConclusionSingle-stage arterial switch with aortic arch reconstruction for Taussig-Bing anomaly with aortic arch obstruction had favorable early- and mid-term outcomes. The incidence of right heart outflow tract obstruction is still higher. The optimal operative procedure should be chosen according to the long-term follow-up.

关 键 词：TAUSSIG-BING畸形 主动脉弓部梗阻 右心流出道狭窄 心脏外科手术 

分 类 号：R726.5[医药卫生—儿科]