关键词: 心律失常性右心室发育不良；桥粒；超声心动描记术；心电描记术；心律失常, 心性

Abstract: This review aims to give an update on the pathogenesis， clinical manifestations， and diagnosis of arrhythmogenic right ventricular cardiomyopathy(ARVC). Arrhythmogenic right ventricular cardiomyopathy is mainly an autosomal dominant inherited disease. Classic symptoms include palpitations， cardiac syncope， and aborted cardiac arrest due to ventricular arrhythmias. Diagnosis is based on the presence of major and minor criteria from the Task Force Criteria revised in 2010(TFC 2010)， which includes evaluation of findings from six different diagnostic categories. Imaging is important in ARVC diagnosis， including both echocardiography and cardiac magnetic resonance imaging. Electrocardiograms(ECGs) and signalaveraged ECGs are analyzed for depolarization and repolarization abnormalities. Ventricular arrhythmias are considered a major diagnostic criterion. Family history of ARVC and detection of an ARVCrelated mutation are included in TFC 2010.

Key words: arrhythmogenic right ventricular dysplasia, desmosomes, echocardiography, electrocardiography, arrhythmias, cardiac