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动脉导管未闭及卵圆孔过早闭合,合并二尖瓣闭锁、左心室发育不全、大动脉转位及肺动脉瓣闭锁(作者译)

[Agenesis of the ductus arteriosus Botalli and premature closure of the foramen ovale, in combination with mitral valve atresia, hypoplastic left ventricle, transposition of the great arteries, and pulmonary valve atresia (author's transl)].

作者信息

Leitner G, Hammerer I

出版信息

Pathol Res Pract. 1980 Dec;170(4):410-9. doi: 10.1016/s0344-0338(80)80045-8.

Abstract

Agenesis of the Ductus arteriosus Botalli is a rare anomaly, which is known to occur in combination with truncus arteriosus, tetralogy of Fallot with pulmonary valve agenesis and also in simple tetralogy. The authors report on a case, where neither a ductus nor any remnant of it could be found in a complex heart malformation consisting of premature closure of the Foramen ovale, mitral atresia, hypoplastic left ventricle, transposition of the great arteries, pulmonary valve atresia and hypoplastic pulmonary artery. Speculations as to the pathogenesis of this anomaly presume slowing and eventual arrest of the ductal blood flow as a consequence of closure of the Foramen ovale in early fetal life. Thus total involution and disappearance of the distal part of the sixth aortic arch also on the left side seem conceivable.

摘要

动脉导管未闭是一种罕见的畸形,已知其可与共同动脉干、肺动脉瓣闭锁的法洛四联症以及单纯法洛四联症合并出现。作者报告了一例病例,在由卵圆孔过早闭合、二尖瓣闭锁、左心室发育不全、大动脉转位、肺动脉瓣闭锁和肺动脉发育不全组成的复杂心脏畸形中,未发现动脉导管及其任何残余部分。关于这种畸形发病机制的推测认为,由于胎儿早期卵圆孔闭合,导致导管血流减慢并最终停止。因此,左侧第六主动脉弓远端部分完全退化和消失似乎是可以想象的。

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