心房异构超声诊断及临床分析  

The diagnostic and clinical value of ultrasound in atrial isomerism

作　　者：杨静[1] 丁文虹[1] YANG Jing;DING Wenhong(Depart-ment of Pediatric Cardiovascular Center,Beijing Anzhen Hospital,Capital Medical University,Beijing Institute of Heart,Lung and Blood Vessel Diseases,Beijing 100029,China)

机构地区：[1]首都医科大学附属北京安贞医院-北京市心肺血管疾病研究所儿童心血管病中心,100029

出　　处：《心肺血管病杂志》2018年第12期1074-1078,1083,共6页Journal of Cardiovascular and Pulmonary Diseases

摘　　要：目的:总结心房异构合并心血管畸形的超声心动图诊断方法,同时分析超声识别心房异构合并体、肺静脉连接方式的临床意义。方法:回顾分析2009年至2017年,于我院儿童心血管病中心住院治疗的心房异构患者超声及临床资料,总结分析该类畸形的超声心动图诊断方法及合并心血管畸形的分布特点。超声心动图检查采用剑突下横切面观察脊柱、腹主动脉与下腔静脉/奇静脉的位置关系,剑突下长轴切面显示腔静脉及肝静脉的回流方式,胸骨旁切面显示双侧心房肌结构及心耳结构,同时显示心内合并畸形,胸骨上窝切面扫查腔静脉及肺静脉的回流途径。结果:右房异构的间接超声表现为:剑突下横切面显示下腔静脉与腹主动脉位于脊柱同一侧,下腔静脉位于腹主动脉前方,常合并无脾;直接表现为双侧心耳均为右心耳结构;左房异构的间接超声表现为:剑突下横切面显示下腔静脉肝段缺如,奇静脉/半奇静脉扩张,位于腹主动脉右后/左后方,上行汇入上腔静脉,常合并脾脏形态、数目、位置异常,直接表现为双侧心耳均为左心耳结构。右心房异构患者均合并心血管复杂畸形,大部分为多种畸形同时合并肺血减少型心血管畸形,其中82例(91%)同时合并肺动脉狭窄或肺动脉闭锁;左心房异构中14例(67%)合并心血管复杂畸形,7例合并简单畸形,合并复杂畸形者均同时合并肺动脉狭窄/闭锁。此外,心房异构患者常见合并体和/或肺静脉异常连接。21例左心房异构患者100%合并下腔静脉肝段缺如,奇静脉或半奇静脉扩张并引入上腔静脉;57%右心房异构患者(51/90)合并双侧上腔静脉分别回流至两侧心房。本组右心房异构患者均行单心室系列矫治;7例左心房异构合并简单畸形者行双心室矫治。结论:心房异构常合并心血管复杂畸形,预后差,超声医生对心房异构合并体/肺静脉连接方式的判断对外科�Objective: To summarize the echocardiographic diagnosis of atrial isomerism and cardiovascular malformation, and to analyze the clinical significance of ultrasonic identification of atrial isomerism with different vena cava and pulmonary vein connection. Methods: The ultrasonic and clinical data of the patients with atrial isomerism in-patients in our hospital for 2009-2017 years were reviewed and analyzed. The diagnostic method of echocardiography and the distribution of cardiovascular malformations were analyzed. Echocardiography under spinal transverse section are used to observe the spine, the location of the abdominal aorta and inferior vena or cava/azygos vein, the spinal long axis plane showed vena cava, and hepatic venous reflux, parasternal plane showed bilateral atrial muscle structure and the structure of auricle, at the same time show heart malformations, suprasternal view showed vena cava and pulmonary vein reflux. Results: The indirect signs of ultrasonic manifestations of right atrial isomerism are: the inferior vena cava and the abdominal aorta are located on the same side of the spine, and the inferior vena cava is located in front of the abdominal aorta. The liver often located at the horizontal position and always associated with no splenic syndrome. The direct sign is both auricles are right auricles.The indirect signs of ultrasonic manifestations of left atrial isomerism are: the absence of the hepatic segment of the inferior vena cava, the azygous/semi-azygous vein dilated at the back of the abdominal aorta and ascending to the superior vena cava. The horizontal position of liver is usually associated with abnormal morphology, number and location of spleen. The direct sign is both auricles are left auricles.A total of 111 cases of atrial isomerism were analyzed, including 90 cases of right atrial isomerism and 21 cases of left atrial isomerism.All the patients with right atrial isomerism were complicated with complex cardiovascular malformation, most of which were combined with pulmonary blo

关 键 词：先天性心脏病 心房异构 内脏异位综合征 体-肺静脉连接异常 

分 类 号：R54[医药卫生—心血管疾病]