超声心动图对合并心内膜垫缺损的无顶冠状静脉窦综合征的诊断价值  

Diagnostic Value of Echocardiography in Unroofed Coronary Sinus Syndrome With Endocardial Cushion Defect

作　　者：梁玉[1] 李慕子 王婧金 万琳媛[1] 齐红霞 朱振辉[1] 闫军[2] 孟红[1] LIANG Yu;LI Muzi;WANG Jingjin;WAN Linyuan;QI Hongxia;ZHU Zhenhui;YAN Jun;MENG Hong(Ultrasound Imaging Center,National Center for Cardiovascular Diseases and Fuwai Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100037,China;Pediatric Cardiac Surgical Center,National Center for Cardiovascular Diseases and Fuwai Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,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小儿心脏外科,北京100037

出　　处：《中国循环杂志》2024年第9期889-895,共7页Chinese Circulation Journal

基　　金：中国医学科学临床转化项目(2022-I2M-C&T-B-051)。

摘　　要：目的:总结合并心内膜垫缺损(ECD)的无顶冠状静脉窦综合征(UCSS)的超声心动图特点,评估超声心动图对其的诊断价值。方法:回顾性分析2017年7月至2022年5月于我院行外科手术治疗的43例合并ECD的UCSS患者的超声心动图结果,并与术中所见对比,评估其诊断准确度。结果:根据Kirklin和Barratt-Boyes分型,43例患者中,Ⅰ型26例(60.5%),Ⅱ型9例(20.9%),Ⅲ型2例(4.7%),Ⅳ型4例(9.3%),Ⅲ型合并Ⅳ型2例(4.7%)。合并部分型ECD 26例(60.5%)、过渡型ECD 7例(16.3%)、完全型ECD10例(23.3%),22例(51.2%)同时合并单心房。27例(62.8%)合并永存左上腔静脉(PLSVC)。合并其他复杂畸形包括:右心室双出口2例、肺动脉闭锁1例、完全型肺静脉异位引流1例、无脾综合征1例。合并简单畸形包括:继发孔型房间隔缺损4例、室间隔缺损2例、动脉导管未闭3例、卵圆孔未闭6例。合并其他异常包括:下腔静脉肝段缺如5例、右上腔静脉发育不良1例、右上腔静脉缺如1例、三房心3例、单发左位心1例、镜面右位心1例、右位主动脉弓4例。43例患者中,术前超声心动图正确诊断30例(69.8%),术中探查确诊13例(30.2%)。其中1例误诊为下腔静脉窦型房间隔缺损,且漏诊PLSVC;12例漏诊UCSS,其中3例同时漏诊PLSVC。结论:合并ECD的UCSS超声心动图诊断难度大,需加强对该畸形胚胎发育及病理解剖特征的认识,提高诊断准确度。Objectives:To evaluate the diagnostic value of echocardiography in unroofed coronary sinus syndrome(UCSS) with endocardial cushion defect(ECD).Methods:The echocardiographic data of 43 patients of UCSS with ECD who underwent surgical treatment in our hospital from July 2017 to May 2022 were retrospectively analyzed.The diagnostic accuracy was evaluated by comparing the echocardiographic findings with the intraoperative exploration results.Results:According to Kirklin and Barratt-Boyes classification,there were 26 cases of type Ⅰ(60.5%),9 cases of type Ⅱ(20.9%),2 cases of type Ⅲ(4.7%),4 cases of type Ⅳ(9.3%),and 2 cases of type Ⅲ combined with type Ⅳ(4.7%).There were 26 cases(60.5%) of partial ECD,7 cases(16.3%) of intermediate ECD,10 cases(23.3%) of total ECD.Twenty two cases(51.2%) were associated with single atrium.Twenty seven cases(62.8%) were associated with persistent left superior vena cava(PLSVC).Other coexisting complicated malformations were as follows:2 cases of double outlet of right ventricle,1 case of pulmonary atresia,1 case of total anomalous pulmonary venous connection,and 1 case of aplenia syndrome.The coexisting simple malformations included 4 cases of ostium secundum atrial septal defect,2 cases of ventricular septal defect,3 cases of patent ductus arterial,and 6 cases of patent foramen ovale.Other abnormalities included 5 cases of absence of hepatic segment of inferior vena cava,1 case of hypoplasia of right superior vena cava,1 case of absence of right superior vena cava,3 cases of cor triatriatum,1 case of isolated levocardia,1 case of mirror image dextrocardia,4 cases of right aortic arch.Of the 43 patients,30(69.8%) were correctly diagnosed by preoperative echocardiography and 13(30.2%) by intraoperative exploration.UCSS was misdiagnosed as inferior vena cava type sinus septal defect and PLSVC was missed in 1 case.UCSS was missed in 12 cases,and PLSVC was missed in 3 cases of them.Conclusions:Diagnosis UCSS with ECD by echocardiography is valuable and challenging.It is necessar

关 键 词：超声心动描记术 无顶冠状静脉窦综合征 心内膜垫缺损 先天性 

分 类 号：R54[医药卫生—心血管疾病]