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肥厚型梗阻性心肌病伴广泛心肌纤维化:尸检病例报告

[Hypertrophic obstructive cardiomyopathy with extensive myocardial fibrosis: case report with autopsy].

作者信息

Hirama M, Yasuda H, Sakamoto S, Kudo T, Ando J

出版信息

Hokkaido Igaku Zasshi. 1985 Mar;60(2):195-205.

PMID:4039700
Abstract

An autopsy case of hypertrophic obstructive cardiomyopathy with extensive myocardial fibrosis is reported in a 43-year-old male. His mother died suddenly at 55. At the age of 39 the patient felt fatigue and feverish sensation followed by dyspnea and palpitation on exertion. He responded to beta-blocker and was discharged on the 51st hospital day. He died suddenly during his work three years and one month after discharge. The heart weighs 700 g. The thickness of the ventricular septum measures up to 3.2 cm, and that of the left ventricular posterior wall 2.2 cm. Subaortic endocardium is moderately thickened. Many patchy fibroses of various sizes and broad linear fibroses are mainly observed in the ventricular septum and in the left ventricular free wall. Microscopic examination shows severe fascicular disarray of hypertrophied myocardial fibers in the ventricular septum and in a part of the left ventricular anterior wall. Pericardial fibrosis, granulation tissue with many capillaries, and slight lymphocytic infiltrate are also noted. These findings suggest that the patient have both congenital hypertrophic cardiomyopathy and myocarditis. There are following possibilities as regards the relation between the two: first, haphazard association of cardiomyopathy with myocarditis; secondly, myocarditis triggered the onset or progression, or both, of cardiomyopathy. He also had liver cirrhosis, probably alcoholic, which appears to accelerate the progression of myocardial disarray and fibrosis.

摘要

报告了一例43岁男性肥厚型梗阻性心肌病合并广泛心肌纤维化的尸检病例。他的母亲在55岁时突然去世。39岁时,患者感到疲劳和发热,随后在劳累时出现呼吸困难和心悸。他对β受体阻滞剂有反应,并在住院第51天出院。出院三年零一个月后,他在工作期间突然死亡。心脏重700克。室间隔厚度达3.2厘米,左心室后壁厚度为2.2厘米。主动脉瓣下内膜中度增厚。主要在室间隔和左心室游离壁观察到许多大小不一的片状纤维化和广泛的线性纤维化。显微镜检查显示室间隔和部分左心室前壁肥厚的心肌纤维严重束状紊乱。还注意到心包纤维化、有许多毛细血管的肉芽组织和轻微的淋巴细胞浸润。这些发现表明该患者既有先天性肥厚型心肌病又有心肌炎。关于两者之间的关系有以下几种可能性:第一,心肌病与心肌炎的偶然关联;第二,心肌炎引发了心肌病的发作或进展,或两者兼而有之。他还患有肝硬化,可能是酒精性的,这似乎加速了心肌紊乱和纤维化的进展。

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