Arrhythmogenic left ventricular cardiomyopathy

被引：4

作者：

Mirmomen, Seyedeh Mojdeh ^{[1
]}

Bradley, Andrew Jay ^{[1
]}

Arai, Andrew Ernest ^{[1
]}

Sirajuddin, Arlene ^{[1
]}

机构：

[1] NHLBI, NIH, Bldg 10, Bethesda, MD 20892 USA

来源：

BJR CASE REPORTS | 2020年 / 6卷 / 01期

关键词：

DILATED CARDIOMYOPATHY; MYOCARDIAL FAT; MUTATION;

D O I：

10.1259/bjrcr.20190079

中图分类号：

R8 [特种医学]; R445 [影像诊断学];

学科分类号：

1002 ; 100207 ; 1009 ;

摘要：

Arrhythmogenic ventricular cardiomyopathy AVC is a heritable heart muscle disorder characterized by fibrofatty infiltration of the myocardium. Intramyocardial fat deposition is considered arrhythmogenic and predisposes patients to life-threatening arrhythmias and sudden cardiac death. The classic subtype of AVC is characterized by fibrofatty replacement of the right ventricular myocardium (i.e. arrhythmogenic right ventricular cardiomyopathy). In advanced cases of arrhythmogenic right ventricular cardiomyopathy, the Ieft ventricle may be involved as well. Predominantly left ventricular involvement by AVC is exceedingly rare and lack of specific diagnostic criteria as well as its potential cardiotoxic effect make its diagnosis challenging and of high importance.

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页数：4

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