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肥厚型心肌病:早期。

Hypertrophic cardiomyopathy: the early years.

机构信息

Harvard Medical School, Boston, MA, USA.

出版信息

J Cardiovasc Transl Res. 2009 Dec;2(4):341-8. doi: 10.1007/s12265-009-9128-3. Epub 2009 Oct 7.

Abstract

Hypertrophic obstructive cardiomyopathy (HOCM) has four major features: (1) severe left ventricular hypertrophy, often most prominent in the basal interventricular septum; (2) frequent familial occurrence with autosomal dominant transmission; (3) occurrence of sudden cardiac death that is usually considered to be due to ventricular fibrillation; and (4) presence of hemodynamic evidence of labile intraventricular obstruction. The key papers describing the recognition of each of these features, as well as of various combinations of them, are reviewed in this paper. Particular attention is focused on the very frequent finding of marked lability of intraventricular obstruction. The recognition of this fourth and last major feature in 1959 makes 2009 the golden anniversary year marking completion of the description of the major features of HOCM.

摘要

肥厚型梗阻性心肌病(HOCM)有四大特征:(1)严重的左心室肥厚,常以室间隔基底部最为显著;(2)常以常染色体显性遗传形式发生的家族性疾病;(3)发生通常认为是由于心室颤动导致的心脏性猝死;(4)存在血流动力学证据的不稳定的室间隔梗阻。本文对描述这些特征(以及它们的各种组合)的主要文献进行了回顾。特别关注于室间隔梗阻明显多变性的这种非常常见的发现。1959 年对第四个也是最后一个主要特征的认识标志着 HOCM 主要特征描述完成的金禧年。

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