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致心律失常性右室心肌病(ARVC)是一种原发性心肌病,其特征性病理改变为心肌细胞变性退化被纤维脂肪组织所替代,最终导致心力衰竭,心律失常,猝死。ARVC是目前青年人及运动员猝死的主要死因之一,其发病率约为1/1000-1/5000,男性较女性多见.近年来越来越多研究表明ARVC与基因突变相关,尤其是编码桥粒蛋白基因,但具体发病机制仍不明确。
Cardiac arrhythmogenic right ventricular cardiomyopathy (ARVC) is a primary cardiomyopathy whose characteristic pathological changes are the degeneration of cardiomyocytes degenerated by fibrous adipose tissue, eventually leading to heart failure, arrhythmia and sudden death. ARVC is one of the main causes of death among young people and athletes at present, the incidence is about 1 / 1000-1 / 5000, more common in men than women.Recently more and more studies have shown that ARVC is related to gene mutation, especially coding Desmoplakin gene, but the specific pathogenesis is still not clear.