致心律失常性右心室心肌病

Arrhythmogenic right ventricular cardiomyopathy

致心律失常性右心室心肌病是以右心室心肌被脂肪或纤维脂肪组织取代为特征的一种原因尚不明确的心肌病。目前已公认是年轻人猝死的重要原因之一。ARVC最显著的病理学特征是弥散性或节段性的右心室游离壁心肌的缺失及纤维脂肪组织的替代,从组织形态学可将ARVC分为两型:(1)单纯脂肪型;(2)纤维脂肪型。与纤维脂肪组织取代右心室心肌有关的发病机制:(1)细胞凋亡或程序性细胞死亡理论;(2)炎症理论;(3)心肌发育不良理论;(4)肌细胞转分化理论。

Arrhythmogenic right ventricular cardiomyopathy(ARVC)is a heart muscle disease of unknown etiology characterized by fatty or fibrofatty infiltration of the right ventricular myocardium.It is well known that ARVC is one of the important causes of sudden death in young individuals.The significant pathological change of ARVC is atrophy and fibrofatty myocardial replacement of the right ventricular free wall.According to the histopathological substrate ARVC could be divided into2types:fatty and fibrofatty myocardial replacement.Possible etiology in-cluding:(1)apoptosis or programmed cell death;(2)inflammatory;(3)myocardium dysplasia and(4)myocyte trans-differentiation theory.