致心律失常性右室心肌病的研究进展

Research Progress of Arrhythmogenic Right Ventricular Cardiomyopathy

致心律失常性右室心肌病(ARVC)是以右室心肌不同程度的被脂肪组织或纤维组织替代为特征的一种常染色体遗传性心肌病。临床上常表现为室性心律失常,进行性心力衰竭,甚至心源性猝死。有时起病隐匿,无症状患者于尸检后才作出诊断,也有部分患者以猝死为首发症状。临床上主要靠心电图,电生理学检查,以及影像学检查提供诊断依据,但心内膜心肌活检仍是确诊的金标准。ARVC治疗包括抗心律失常药物,置入埋藏式心律转复除颤器(ICD),导管射频消融以及心脏移植。

AArrhythmogenic right ventricular cardiomyopathy （ARVC） is an autosomal hereditary cardiomyopathy which is characterized by progressive fi brofatty replacement of right ventricular myocardium. The clinical manifestation include ventricular arrhythmias, progressive hear failure, and even sudden cardiac death. While the stage of onset is often atypical, some patients were determined by autopsy or expressed sudden cardiac death as the initial symptom. The diagnostic evidence are based on the electrocardiogram, electrophysiological examination and imaging examination, of which endomyocardial biopsy is the gold standard for diagnosis. The treatment consists of anti-arrhythmic drugs, implantable cardioverter defi brillator, catheter ablation, and heart transplant.