胎儿心房异构及其合并心内外畸形的病理学分析  

The pathologic analyses of atrial isomerism accompanied with cardiac anomalies of fetus

作　　者：商建峰[1] 贺晨宇 陈东[1] 方微[1] 武迎[1] 崔亚艳[1] 滕飞[1] 付稳[1] 董方 于玮[1] 李倩 朱庆[1] 

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病理科,100029 [2]郑州大学基础医学院

出　　处：《心肺血管病杂志》2018年第1期1-4,共4页Journal of Cardiovascular and Pulmonary Diseases

基　　金：首都医科大学附属北京安贞医院院长科技发展基金课题资助(2016F01)

摘　　要：目的:对胎儿心房异构畸形进行尸体解剖分析,提高该疾病诊断的精准性。方法:回顾分析我院近几年,在怀孕期间经超声心动图畸形筛查,发现先天性心脏畸形引产而进行尸体解剖病例,检查胎儿心房结构、心脏畸形、静脉畸形以及内脏的畸形情况。结果:胎儿先天性心脏病尸体解剖391例,心房异构17例,男性9例,女性8例,其中右心房异构12例。伴发主要心内畸形:永存左上腔静脉8例,房间隔缺损7例,肺动脉闭锁6例,单心室6例,肺静脉畸形9例,右位主动脉弓5例;伴发心外畸形:脾缺如12例,腹腔内脏位置异常8例;左心房异构5例,伴发主要心内畸形:下腔静脉中断5例,伴发心外畸形:多脾4例,肺发育异常3例,腹腔内脏位置异常2例。结论:胎儿右心房异构多于左心房异构,右心房异构常合并严重而复杂心脏畸形。Objective： To improve the accuracy diagnosis of atrial isomerism by analyzing characteristics of atrial isomerism autopsy cases of fetus. Methods： All reviewed 391 autopsy cases of congenital heart diseases were examined by echocardiography during pregnancy. The autopsy process included checking the cardiac abnormalities,the vessel malformations,and the deformity of the internal organs. Results： There were 17（ 4. 345%） cases of atrial isomerism in 391 cases of fetal congenital heart disease including 12（ 3. 017%） patients in the right atrial isomerism group and left in 5（ 1. 238%） patients. In the right atrial isomerism group,there were 8 cases with persistent left superior vena cava,7 cases with atrial septal defect,6 cases with pulmonary atresia,9 cases with pulmonary venous malformation,6 cases with single ventricle,5 cases with right aortic arch and 12 cases with asplenia. In the right atrial isomerism group,there were 5 cases with interruption of inferior vena cava and 4 cases with polysplenia. Conclusion：（1）Patients with atrial isomerism is often accompanied with complex and severe cardiac malformations.（2）The higher prevalence（ 3. 017%） of right atrial isomerism was found in our study.

关 键 词：先天性心脏病 心房异构 内脏异位综合征 胎儿 

分 类 号：R54[医药卫生—心血管疾病]