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一例罕见的因存在心室内肌性隔而导致的孤立性先天性右心室流入道梗阻病例。

A rare case of isolated congenital right ventricular inflow obstruction due to the presence of an intraventricular muscular shelf.

作者信息

Nanna Giovanni J, Nanna Michael G, Muchnik Danielle, DeRose Joseph J, Narasimhan Seshasayee

机构信息

Sackler School of Medicine, New York, New York 10065, USA.

出版信息

Tex Heart Inst J. 2012;39(4):557-9.

Abstract

A 56-year-old man presented with anasarca and a 40-lb weight gain that had occurred over the course of 3 to 4 weeks. He had a history of permanent atrial fibrillation and a congenital anomaly of the right ventricular inflow tract. This defect consisted of a muscular shelf in the right ventricular inflow tract, which encased the tricuspid subvalvular apparatus in such a manner that it created tricuspid stenosis. The clinical consequences of this anatomic and hemodynamic situation were a massively dilated right atrium, permanent atrial fibrillation, and clinical evidence of right-sided heart failure, including fluid retention and ascites. The patient underwent surgical resection of the muscular shelf, which was followed by progressive resolution of the ascites and fluid retention.

摘要

一名56岁男性出现全身水肿,体重在3至4周内增加了40磅。他有永久性心房颤动病史和右心室流入道先天性异常。该缺陷表现为右心室流入道内的一个肌性嵴,它以包裹三尖瓣瓣下结构的方式造成了三尖瓣狭窄。这种解剖和血流动力学状况的临床后果是右心房极度扩张、永久性心房颤动以及右侧心力衰竭的临床证据,包括液体潴留和腹水。患者接受了肌性嵴的手术切除,随后腹水和液体潴留逐渐消退。

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