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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。
PURPOSE: Transposition of the great arteries (TGA) is a rare complication of congenital heart deformity. Its classification and conformation are rather complicated. The TGA of pure complete type has a very poor natural prognosis and a very high case fatality rate. It is a serious adverse effect on fetal health Of congenital heart disease, so a correct understanding of its pathological anatomy is the fundamental guarantee for the diagnosis and treatment of TGA. Methods: A retrospective review of our hospital from 2006 to date, anatomical specimens of fetal cadavers and their pathological anatomy text file data of 174 cases, in line with TGA diagnosis of 29 cases, the clinical data were analyzed to observe the pathological anatomy of its morphology Features and ultrasound imaging findings were compared to summarize the various types of TGA anatomical morphological characteristics, to find out the main TGA pathological anatomy of the operation. TGA accounted for 29.7% (16.7%) in 1174 fetuses. According to the morphological classification, 12 TGA cases accounted for 41.4% of TGA cases, and 17 TGA cases accounted for 58.6%. Among them, 11 cases (91.7%) of pure TGA in complete TGA and 1 case of functional TGA (8.3%). Partial TGA, right ventricular double outlet in 15 cases (88.2%) (Taussig-Bing deformity in 7 cases); left ventricular double outlet in 2 cases (11.8%); 3 according to the segmental analysis of heart, SDD 25 cases of this study, SDL 2 cases, 2 cases of SLL. The major complications of 4TGA were as follows: 19 cases of ventricular septal defect, 10 cases of pulmonary arterial deformity and 8 cases of aortic malformation. 5TGA associated with syndrome: 21-trisomy syndrome, splenomegaly and so on. Conclusion: The typing of TGA is more complicated. During the autopsy, the TGA with abnormal atrioventricular junction should pay more attention to determine the atrial and ventricular connection status and the location of the two aorta to ensure the correct classification of TGA. Ventricular septal defect and other cardiac malformations exist in the survival of children with effective blood circulation compensation, correct search, analysis of TGA merger deformity is the prerequisite for the diagnosis and treatment programs, but also to study the molecular basis of TGA molecular pathogenesis of morphological basis .