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自1985年~1997年围产儿死亡进行尸体解剖623例,发现先天畸形155例,其中先心畸形32例,占先天畸形20.6%,位居第二位。先心畸形的种类:单一畸形13例占40.6%,心内复杂畸形17例占53.1%。发现2例极罕见先天心脏原发肿瘤。伴心外畸形16例占50%。先心畸形多种多样,类型各不一样,尽管如此还是能分出一个主要的异常及循环中哪处发育障碍。本组先心畸形中以心内复杂畸形多见,由于心内复杂畸形病死率高,临床上短期内确诊及在新生儿期的治疗难度大,往往早期夭折。现公认为先心畸形可能单基因或多基因或染色体遗传病。
From 1985 to 1997 perinatal death of 623 cases of autopsy, found 155 cases of congenital malformations, including 32 cases of congenital malformations, congenital malformations accounted for 20.6%, ranking second. Types of congenital malformations: a single deformity in 13 cases accounted for 40.6%, 17 cases of intracardiac malformations accounted for 53.1%. 2 cases found extremely rare congenital heart tumor. 16 cases with extrahepatic malformations accounted for 50%. There are many different forms of preocular deformity and different types of neovascularization. However, it is still possible to distinguish between a major anomaly and the developmental disorder in the circulation. The group of heart deformity in the heart complex deformity more common, due to complex intra-cardiac deformity mortality, clinically diagnosed in the short term and neonatal treatment is difficult, often early death. It is recognized that congenital malformations may be single or polygenic or chromosomal genetic disease.