目的　明确先天性心血管系统畸形 (简称先心 )对儿童 ,特别是围生儿致死的意义。方法　在全面分析 163例先心病病理标本的基础上 ,重点对 82例致死性先心进行分析。结果　先心的尸检检出率为 2 1.7% (占先天性畸形的 5 0 .3 % ) ,其中致死性先心占 5 0 .3 % (82 / 163 ) ,依次为婴儿型主动脉缩窄、半心畸形、永存动脉干、大动脉转位及心内膜垫缺损等。结论　先心是围生儿的第一死因 ,小儿年龄越小 ,检出率越高 Objective To clarify the congenital cardiovascular system deformity (referred to as congenital heart) on children, especially the meaning of perinatal death. Methods Based on a comprehensive analysis of 163 pathological specimens of congenital heart disease, 82 fatal cases were analyzed emphatically. Results The rate of congenital autopsy was 27.7% (accounting for 50.3% of congenital malformations), of which fatal congenital heart disease accounted for 53.3% (82/163), followed by infantile aortic constriction , Half heart-shaped deformity, eternal trunk artery, aortic transposition and endocardial cushion defect. Conclusions The first heartache is the first cause of perinatal death, the younger the child, the higher the detection rate