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左右心室心肌壁部分先天性发育不全。两例羊皮纸样心脏综合征患者的临床及尸检观察

Congenital hypoplasia of portions of both right and left ventricular myocardial walls. Clinical and necropsy observations in two patients with parchment heart syndrome.

作者信息

Waller B F, Smith E R, Blackbourne B D, Arce F P, Sarkar N N, Roberts W C

出版信息

Am J Cardiol. 1980 Nov;46(5):885-91. doi: 10.1016/0002-9149(80)90444-0.

DOI:10.1016/0002-9149(80)90444-0
PMID:7435401
Abstract

Clinical and morphologic findings are described in two patients with congenital hypoplasia of portions of both right and left ventricular free walls in the absence of associated coronary or valvular heart disease. One, a 61 year old man who had never had clinical evidence of cardiac dysfunction, died suddenly and unexpectedly. The second, a 55 year old woman, died of progressive, eventually intractable congestive heart failure of 29 months' duration. Although at least 22 necropsy patients have previously been reported to have "parchment-like" thinning of portions of the right ventricular free wall, only one patient has previously been described with such thinning of portions of both right and left ventricular free walls. The spectrum of right or right and left ventricular wall congenital hypoplasia is a broad one, with nearly half of described patients dying of congestive heart failure in the 1st year of life and the other half reaching adulthood with or without manifestations of cardiac dysfunction.

摘要

本文描述了两名左右心室游离壁部分先天性发育不全患者的临床和形态学表现,患者无相关冠状动脉或瓣膜性心脏病。其中一名61岁男性,既往无心脏功能障碍的临床证据,突然意外死亡。另一名55岁女性,死于持续29个月的进行性、最终难以治疗的充血性心力衰竭。尽管此前至少有22例尸检患者被报道右心室游离壁部分有“羊皮纸样”变薄,但此前仅有1例患者被描述为左右心室游离壁部分均有此类变薄。右心室或左右心室壁先天性发育不全的范围很广,近一半的已描述患者在1岁时死于充血性心力衰竭,另一半成年后有或无心脏功能障碍表现。

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Congenital hypoplasia of portions of both right and left ventricular myocardial walls. Clinical and necropsy observations in two patients with parchment heart syndrome.左右心室心肌壁部分先天性发育不全。两例羊皮纸样心脏综合征患者的临床及尸检观察
Am J Cardiol. 1980 Nov;46(5):885-91. doi: 10.1016/0002-9149(80)90444-0.
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[Myocardial dysplasia of the right ventricle (Uhl's anomaly) as a possible cause of sudden, unexpected death].[右心室心肌发育异常(乌尔氏畸形)作为突然意外死亡的可能原因]
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