From the Department of Medicine, Cardiology Division, New York Medical College/Westchester Medical Center, Valhalla, NY.
Cardiol Rev. 2020 Nov-Dec;28(6):319-324. doi: 10.1097/CRD.0000000000000292.
Arrhythmogenic right ventricular cardiomyopathy, formerly called "arrhythmogenic right ventricular dysplasia," is an under-recognized clinical entity characterized by ventricular arrhythmias and a characteristic ventricular pathology. Diagnosis is often difficult due to the nonspecific nature of the disease and the broad spectrum of phenotypic variations. Therefore, consensus diagnostic criteria have been developed which combine electrocardiographic, echocardiographic, cardiac magnetic resonance imaging and histologic criteria. In 1994, an international task force first proposed the major and minor diagnostic criteria of arrhythmogenic right ventricular cardiomyopathy based on family history, arrhythmias, electrocardiographic abnormalities, tissue characterization, and structural and functional right ventricular abnormalities. In 2010, the task force criteria were revised to include quantitative abnormalities. These diagnostic modalities and the most recent task force criteria are discussed in this review.
致心律失常性右室心肌病，以前称为“致心律失常性右室发育不良”，是一种临床认识不足的疾病，其特征为室性心律失常和特征性的心室病理学改变。由于该病的非特异性和表型变异的广泛范围，诊断通常较为困难。因此，已经制定了共识诊断标准，这些标准结合了心电图、超声心动图、心脏磁共振成像和组织学标准。1994 年，一个国际工作组首次根据家族史、心律失常、心电图异常、组织特征以及结构和功能右心室异常提出了致心律失常性右室心肌病的主要和次要诊断标准。2010 年，工作组标准进行了修订，纳入了定量异常。本综述讨论了这些诊断方法和最新的工作组标准。
