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利物浦的先天性心脏病:1960 - 1969年

Congenital heart disease in Liverpool: 1960--69.

作者信息

Kenna A P, Smithells R W, Fielding D W

出版信息

Q J Med. 1975 Jan;44(173):17-44.

PMID:125433
Abstract

The incidence of congenital heart disease (C.H.D.) in Liverpool from 1960 to 1969 inclusive has been determined from the Liverpool Congenital Abnormalities Registry with a follow-up period of 3 to 12 years. The incidence is 6-6 per 1000 total births and this probably represents a very small degree of under-reporting. There is no consistent seasonal variation in the incidence of any of the main congenital heart lesions. In general, infants with C.H.D. tend to be of lower birth weight and born after shorter gestation than controls. This is most conspicuous with patent ductus arteriosus (P.D.A.). Females preponderate in P.D.A. and males in transposition. There is probably also a male preponderance in coarctation and aortic stenosis. Fallot's tetralogy is associated with increased maternal age and parity. Pregnancies leading to the birth of a baby with C.H.D. are complicated by threatened abortion more frequently than are controls. The concordance rate for C.H.D. in twins is low. Monozygotic twins are more liable to C.H.D. than are dizygotic twins. The incidence of C.H.D. in the siblings of affected propositi is 2-3 times that expected. Affected sibs often have the same lesion. About 20 per cent of infants with C.H.D. have associated major defects notably monogolism and defects of the alimentary, skeletal, genito-urinary and nervous systems. These are responsible for the early death of about one quarter of all infants born with C.H.D. The data presented here suggest that environmental rather than genetic factors are predominantly responsible for congenital heart disease.

摘要

1960年至1969年(含)利物浦地区先天性心脏病(C.H.D.)的发病率已根据利物浦先天性异常登记处的数据确定,随访期为3至12年。发病率为每1000例总出生数中有6.6例,这可能仅代表极低程度的报告不足。任何主要先天性心脏病变的发病率均无一致的季节性变化。一般来说,患有先天性心脏病的婴儿往往出生体重较低,孕期较短。这在动脉导管未闭(P.D.A.)中最为明显。动脉导管未闭中女性居多,而大血管转位中男性居多。缩窄和主动脉狭窄可能也以男性居多。法洛四联症与母亲年龄和产次增加有关。导致患有先天性心脏病婴儿出生的妊娠比对照组更常并发先兆流产。双胞胎中先天性心脏病的一致率较低。单卵双胞胎比双卵双胞胎更易患先天性心脏病。受影响先证者的兄弟姐妹中先天性心脏病的发病率是预期发病率的2至3倍。受影响的兄弟姐妹往往患有相同的病变。约20%的先天性心脏病婴儿伴有主要缺陷,尤其是唐氏综合征以及消化、骨骼、泌尿生殖和神经系统缺陷。这些缺陷导致约四分之一患有先天性心脏病的婴儿早期死亡。此处呈现的数据表明,先天性心脏病主要由环境因素而非遗传因素导致。

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