内脏异位综合征全腔静脉-肺动脉连接术前的超声心动图诊断  

The Method and Value of Echocardiography in the Diagnosis of Patients With Heterotaxy Syndrome Before Total Cavopulmonary Connection

作　　者：李慧[1] 贾玉和[2] 孟红[1] 李慕子 王强[1] 逄坤静[1] 王剑鹏[1] LI Hui;JIA Yuhe;MENG Hong;LI Muzi;WANG Qiang;PANG Kunjing;WANG Jianpeng(Department of Ultrasound,National Center for Cardiovascular Diseases and Fuwai Hospital,CAMS and PUMC,Beijing 100037,China)

机构地区：[1]中国医学科学院,北京协和医学院,国家心血管病中心,阜外医院,超声影像中心,北京市100037 [2]中国医学科学院,北京协和医学院,国家心血管病中心,阜外医院,心律失常中心,北京市100037

出　　处：《中国循环杂志》2020年第9期900-903,共4页Chinese Circulation Journal

摘　　要：目的:探讨超声心动图在内脏异位综合征患者全腔静脉-肺动脉连接术(TCPC)前的诊断方法及应用价值。方法:回顾性分析自2011年6月至2019年6月在我院行TCPC的内脏异位综合征患者32例,其中左心房异构(多脾综合征)20例,右心房异构(无脾综合征)12例。收集患者超声心动图检查结果以及手术和临床资料。结果:内脏异位综合征患者心脏位置异常比例为50.0%,多脾综合征及无脾综合征患者单心室分别为12例(60.0%)和9例(75.0%);2例(10.0%)多脾综合征患者存在部分性肺静脉异位引流,4例(33.3%)无脾综合征患者发生完全性肺静脉异位引流;内脏异位综合征患者经常合并体静脉异位引流,8例(40.0%)多脾综合征患者下腔静脉肝段缺如;10例(50.0%)多脾综合征及5例(41.7%)无脾综合征患者存在肝静脉异位引流。12例患者行分期手术,全部患者均行TCPC。结论:内脏异位综合征患者心内畸形及体静脉、肺静脉连接复杂,TCPC前超声心动图诊断对指导治疗作用重大。Objectives:To explore the method and value of echocardiography in the diagnosis of patients with heterotaxy syndrome before total cavopulmonary connection(TCPC).Methods:A total of 32 patients undergoing TCPC from June 2011 to June 2019 in our hospital were reviewed retrospectively,including 20 with left atrial isomerism(polysplenia syndrome)and 12 with right atrial isomerism(asplenia syndrome).The results of echocardiography,surgical and clinical data were collected and analyzed.Results:The proportion of heterotaxy syndrome with abnormal heart location was about 50.0%.The polysplenia and asplenia syndrome with single ventricle was 12(60.0%)and 9 cases(75.0%),respectively.Anomalous pulmonary venous drainage was observed in 2(10.0%)patients with polysplenia,and total anomalous pulmonary venous drainage occurred in 4(33.3%)patients with asplenia syndrome.Patients with heterotaxy syndrome were often associated with anomalous vena cava drainage.8 cases(40.0%)of the patients with polysplenia syndrome had absence of hepatic inferior vena cava.Ten patients(50.0%)with polysplenia syndrome and 5 cases(41.7%)with asplenia syndrome had separate hepatic venous.One stage operation was performed in 12 patients and all underwent TCPC in the second stage.Conclusions:Patients with heterotaxy syndrome are often associated with complicated congenital heart diseases,anomalous vena cava and pulmonary venous drainage.Echocardiography has an important clinical value in preoperative diagnosis for patients assigned to TCPC.

关 键 词：内脏异位综合征 多脾综合征 无脾综合征 全腔静脉-肺动脉连接术 超声心动图 

分 类 号：R54[医药卫生—心血管疾病]