胎儿大动脉转位尸检标本的临床病理学分析  

Clinicopathologic analysis of the autopsy specimens of transportation of great arteries

作　　者：方微[1] 陈东[1] 商建峰[1] 武迎[1] 滕飞[1] 连国亮[1] 梅少帅[1] 石凤茹[1] 李彦玮[1] 韩丽媛[1] 王伟[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

出　　处：《心肺血管病杂志》2014年第6期866-869,880,共5页Journal of Cardiovascular and Pulmonary Diseases

基　　金：北京市优秀人才资助项目(2012A003C34000001);首都医科大学基础临床科研合作基金课题(14JL56)

摘　　要：目的:大动脉转位(TGA)是一种并非少见的复杂性先天性心脏畸形,分型及构象较复杂,单纯性完全型TGA自然预后极差,病死率极高,是一种严重影响胎儿健康的先天性心脏病,因此正确认识其病理解剖形态是诊断及治疗TGA的根本保证。方法:回顾性复习我院2006年至今,胎儿尸体解剖标本及其病理解剖学文字档案资料174例,符合TGA诊断的有29例,分析其临床资料,观察其病理解剖学特点,对其形态学特征与超声影像学等检查结果进行对比,总结归纳各型TGA的解剖形态学特征,找出进行TGA病理学解剖的操作要点。结果:1174例胎儿尸体解剖标本中,TGA占29例(16.7%);2根据形态学分类:完全型TGA 12例,占TGA例数的41.4%,部分型TGA 17例,占58.6%。其中,完全型TGA中单纯性完全型TGA 11例(91.7%),功能矫正型TGA 1例(8.3%)。部分型TGA中,右心室双出口15例(88.2%)(Taussig-Bing畸形7例);左心室双出口2例(11.8%);3根据心脏节段性分析,本研究SDD 25例,SDL 2例,SLL 2例。4TGA主要合并心脏畸形依次为:室间隔缺损19例,肺动脉系畸形10例,主动脉系畸形8例。5TGA伴有综合征:21-三体综合征、多脾综合征等。结论:TGA的分型较复杂,在进行尸体解剖时,对于房室连接异常的TGA更应注意判断心房、心室的连接状态,两根大动脉的位置关系,以保证对TGA的正确分型。室间隔缺损等合并心脏畸形的存在是患儿存活的有效血液循环的代偿,正确寻找、分析TGA合并畸形的类型是决定诊疗方案的前提,也是研究TGA分子遗传学发病机制的的形态学基础。Objective：Transportation of great arteries （TGA） is not a rare complicated congenital heart malformation. It is one of the severe congenital heart diseases because of its complicated subtype and conformation. Simple and completed TGA has a very poor prognosis and high mortality. It is very important to correctly know the pathologic anatomies of TGA. It is the fundamental factor to diagnosis and treatment for TGA. Methods： One hundred and seventy-four fetus autopsy simples and their autopsy pathologic records were reviewed at Anzhen Hospital from 2006 year to now. There were 29 cases diagnosed with TGA. The clinical features of all cases were reviewed. The pathologic anatomic features of TGA cases were checked and observed again. All these anatomic features of each case were compared with its Echocardiography （UCG） descriptive diagnosis. The anatomic features all kinds of subtypes of TGA were analyzed. Operation points of autopsy for TGA cases were summarized. Results： （1） there were 29 cases （16. 7% ） TGA cases of all 174 cases fetus autopsy simples; （2）Aeeording to the anatomy, there were 12 cases （41.4%） of completed TGA（cTGA） , 17 cases （58. 6% ） of partial TGA （pTGA）. Among cTGA, there were llcases （91.7%） of simple subtypes; lcase （8. 3% ） of functional corrected TGA. Among pTGA, there were 15 cases （88.2%） of double outlet right ventricle（DORV） ; 2 cases （ 11.8% ） of double outlet left ventricle （DOLV） ; （3） According to the segmental analysis of heart, there were 25 cases SDD, 2 cases SDL and 2 cases SLL in our study. （4） The main accompanied heart malformations of TGA were 19 cases VSD, 8 cases pulmonary artery lesions and aortic lesions; （5） TGA accompanied syndromes： 1 case trisomy 21 and 1 case polysplenia syndrome. Coneluslon： Correctly judging the connection status of atrium and ventricular, two main arteries, as anti-position of atrioventricular, is the key point for division TGA in autopsy. Accompanied with heart malf

关 键 词：大动脉转位 尸体解剖 病理解剖学特征 分子遗传学发病机制 

分 类 号：R54[医药卫生—心血管疾病]