Abstract：
Arrhythmogenic cardiomyopathy (ACM) is a heart muscle disease characterized by prominent \"non-ischemic\" myocardial scarring predisposing to ventricular electrical instability. Diagnostic criteria for the original phenotype, arrhythmogenic right ventricular cardiomyopathy (ARVC), were first proposed in 1994 and revised in 2010 by an international Task Force (TF). A 2019 International Expert report appraised these previous criteria, finding good accuracy for diagnosis of ARVC but a lack of sensitivity for identification of the expanding phenotypic disease spectrum, which includes left-sided variants, i.e., biventricular (ABVC) and arrhythmogenic left ventricular cardiomyopathy (ALVC). The ARVC phenotype together with these left-sided variants are now more appropriately named ACM. The lack of diagnostic criteria for the left ventricular (LV) phenotype has resulted in clinical under-recognition of ACM patients over the 4 decades since the disease discovery. In 2020, the \"Padua criteria\" were proposed for both right- and left-sided ACM phenotypes. The presently proposed criteria represent a refinement of the 2020 Padua criteria and have been developed by an expert European TF to improve the diagnosis of ACM with upgraded and internationally recognized criteria. The growing recognition of the diagnostic role of CMR has led to the incorporation of myocardial tissue characterization findings for detection of myocardial scar using the late‑gadolinium enhancement (LGE) technique to more fully characterize right, biventricular and left disease variants, whether genetic or acquired (phenocopies), and to exclude other \"non-scarring\" myocardial disease. The \"ring-like\' pattern of myocardial LGE/scar is now a recognized diagnostic hallmark of ALVC. Additional diagnostic criteria regarding LV depolarization and repolarization ECG abnormalities and ventricular arrhythmias of LV origin are also provided. These proposed upgrading of diagnostic criteria represents a working framework to improve management of ACM patients.
摘要：
心律失常性心肌病（ACM）是一种心肌疾病，其特征是明显的“非缺血性”心肌疤痕诱发心室电不稳定性。原始表型的诊断标准，致心律失常性右心室心肌病（ARVC），于1994年首次提出，并于2010年由国际工作队（TF）修订。2019年国际专家报告评估了这些以前的标准，发现对ARVC的诊断具有良好的准确性，但对鉴定不断扩大的表型疾病谱缺乏敏感性，其中包括左侧变体，即，双心室（ABVC）和致心律失常性左心室心肌病（ALVC）。现在将ARVC表型与这些左侧变体一起更恰当地命名为ACM。左心室（LV）表型的诊断标准的缺乏导致ACM患者在疾病发现后的40年中的临床认识不足。2020年，针对右侧和左侧ACM表型提出了“帕多瓦标准”。目前提出的标准代表了2020年帕多瓦标准的改进，并由欧洲TF专家开发，以提高ACM的诊断水平和国际公认的标准。对CMR的诊断作用的日益认识已导致将心肌组织表征结果纳入使用晚期钆增强（LGE）技术检测心肌瘢痕，以更充分地表征正确，双心室和左侧疾病变异，无论是遗传还是获得性（表型），并排除其他“非瘢痕性”心肌疾病。心肌LGE/瘢痕的“环状”模式现在是ALVC的公认诊断标志。还提供了关于LV去极化和复极化ECG异常和LV起源的室性心律失常的附加诊断标准。这些建议的诊断标准升级代表了改善ACM患者管理的工作框架。
