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起源于主动脉瓣左冠窦的主动脉-左心室隧道:先天性主动脉瓣关闭不全的罕见病因

[Aorto-left ventricular tunnel with origin in the left sinus of Valsalva: a rare cause of congenital aortic insufficiency].

作者信息

Ries M, Singer H, Hofbeck M, Buheitel G, von der Emde J

机构信息

Kardiologische Abteilung, Universität Erlangen-Nürnberg.

出版信息

Z Kardiol. 1994 Jul;83(7):519-24.

PMID:7941653
Abstract

Aortico-left-ventricular tunnel is a rare congenital cardiac lesion that often results in severe aortic insufficiency in infancy. Normally, the defect appears immediately above the right coronary sinus of Valsalva and consists of a tunnel-like connection between the aortic root and the left ventricle. The differential diagnosis to coronary artery fistulae and ruptured aneurysms of the sinus of Valsalva is often difficult. We describe a patient with an aortico-left-ventricular tunnel originating from the left aortic sinus. Similar findings have been described in only three case reports. Additionally, in our case there was a stenosis in the midportion of the tunnel which was connected to an intraseptal aneurysm communicating with the left ventricular chamber via a "septal" defect. The infant underwent successful surgical correction of this malformation at 1 year and 5 months of age.

摘要

主动脉左心室通道是一种罕见的先天性心脏病变,常导致婴儿期严重的主动脉瓣关闭不全。正常情况下,缺损位于主动脉窦右冠窦上方,由主动脉根部与左心室之间的隧道样连接组成。与冠状动脉瘘和主动脉窦瘤破裂的鉴别诊断往往很困难。我们描述了一名起源于左主动脉窦的主动脉左心室通道患者。仅有三篇病例报告描述了类似的发现。此外,在我们的病例中,隧道中部存在狭窄,该狭窄与一个通过“室间隔”缺损与左心室腔相通的室间隔瘤相连。该婴儿在1岁5个月时成功接受了这种畸形的手术矫正。

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