Mirmomen Seyedeh Mojdeh, Bradley Andrew Jay, Arai Andrew Ernest, Sirajuddin Arlene
National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
BJR Case Rep. 2020 Feb 12;6(1):20190079. doi: 10.1259/bjrcr.20190079. eCollection 2020 Mar.
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 ( arrhythmogenic right ventricular cardiomyopathy). In advanced cases of arrhythmogenic right ventricular cardiomyopathy, the left 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.
致心律失常性右室心肌病(AVC)是一种遗传性心肌疾病,其特征为心肌纤维脂肪浸润。心肌内脂肪沉积被认为具有致心律失常性,使患者易发生危及生命的心律失常和心源性猝死。AVC的经典亚型特征为右心室心肌纤维脂肪替代(致心律失常性右心室心肌病)。在致心律失常性右心室心肌病的晚期病例中,左心室也可能受累。AVC主要累及左心室极为罕见,缺乏特异性诊断标准以及其潜在的心脏毒性作用使其诊断具有挑战性且极为重要。