致心律失常性右室心肌病的临床诊断特点

Clinical diagnostic characteristics of arrhythmogenic right ventricular cardiomyopathy

致心律失常性右室心肌病(ARVC)是遗传性心肌病的一种,以右心室心肌细胞被纤维、脂肪组织代替为主要病理特征。临床主要表现为反复发生心律失常、心衰和猝死,在年轻人及运动员猝死中具有重要地位。因此,有必要进一步加深对该疾病的病理、生理和临床特征的认识和进一步研究。该文就ARVC的临床特点、心电图表现、超声心动图特点和核磁共振成像特点作一综述。

Arrhythmogenic right ventricular cardiomyopathy （ARVC） is a hereditary cardiomyopathy, which is characterized by the replacement of right ventrieular eardioeytes by fibrous and adipose tissue. The clinical manifes- tations include repeated arrhythmias, heart failure and sudden cardiac death, and with the important status of sudden cardiac death in young people and athletes. Therefore, it is necessary to deepen the recognization and the future re- search about the pathology, pathophysiology and clinical characteristics of ARVC. This article is to review the clinical characteristics, electrocardiogram manifestation, ultrasonic cardiogram and MRI characteristics of ARVC.