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无肥厚型肥厚型心肌病:两个心肌排列紊乱但心肌质量未增加的家系。

Hypertrophic cardiomyopathy without hypertrophy: two families with myocardial disarray in the absence of increased myocardial mass.

作者信息

McKenna W J, Stewart J T, Nihoyannopoulos P, McGinty F, Davies M J

机构信息

Department of Cardiological Sciences, St George's Hospital Medical School, London.

出版信息

Br Heart J. 1990 May;63(5):287-90. doi: 10.1136/hrt.63.5.287.

Abstract

Two families are described in which individuals showed widespread myocardial disarray at histological examination, in the absence of macroscopic cardiac hypertrophy. In one family the clinical presentation was that of sudden unexpected cardiac death in four family members; members of the other family presented with electrocardiographic repolarisation changes and abnormalities of left ventricular diastolic function. The finding of myocardial disarray, the characteristic histological abnormality of hypertrophic cardiomyopathy, in the absence of increased cardiac mass suggests a wider range of abnormality in hypertrophic cardiomyopathy than is currently recognised.

摘要

本文描述了两个家族,其中个体在组织学检查中显示广泛的心肌排列紊乱,但无明显的心脏肥大。在一个家族中,四名家族成员临床表现为意外猝死;另一个家族的成员则表现为心电图复极改变和左心室舒张功能异常。在无心脏质量增加的情况下发现心肌排列紊乱,即肥厚型心肌病的特征性组织学异常,提示肥厚型心肌病的异常范围比目前公认的更广。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0706/1024478/1bb1faa97b6e/brheartj00053-0026-a.jpg

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