Corrado Domenico, Zorzi Alessandro, Bauce Barbara, Rigato Ilaria, Cipriani Alberto, Perazzolo Marra Martina, Pilichou Kalliopi, Basso Cristina, Ungaro Simone, Graziano Francesca
Dipartimento di Scienze Cardio-Toraco-Vascolari e Sanità Pubblica, Università Degli Studi, Padova.
Dipartimento di Scienze Cardio-Toraco-Vascolari e Sanità Pubblica, Università Degli Studi, Padova - Department of Sports Medicine, Semmelweis University, Budapest, Ungheria.
G Ital Cardiol (Rome). 2024 Oct;25(10):735-746. doi: 10.1714/4336.43216.
Arrhythmogenic cardiomyopathy (CMA) is a cardiac disease characterized by non-ischemic ventricular scarring and electrical instability. The diagnosis of CMA still remains challenging today and requires the use of a set of criteria, since no single diagnostic test represents the gold standard. The first diagnostic criteria were defined and disseminated in 1994 and then revised in 2010, focusing mainly on right ventricular involvement. In 2019, an international panel of experts identified the limitations of the previous diagnostic criteria. The 2020 Padua criteria included a specific pathway for the diagnosis of left ventricular variants and emphasized the need for the use of cardiac magnetic resonance imaging in the characterization of myocardial scarring. These criteria were further refined and published in 2023 as European Task Force (TF) criteria, thus gaining international recognition.Exploring the history of CMA and its diagnosis, in this review we analyze the changes and progress in the 20 years that have occurred from the first version of the criteria in 1994 to the latest in European TF of 2023, highlighting the evolution of our knowledge of the pathobiology and morpho-functional characteristics of the disease. One of the most relevant updates is undoubtedly the introduction of the concept of "scarring/arrhythmogenic cardiomyopathy", a definition that enhances the main features of the pathology and emphasizes the multiplicity of phenotypes and clinical presentations independent of etiology.
致心律失常性心肌病（CMA）是一种以非缺血性心室瘢痕形成和电不稳定为特征的心脏疾病。如今，CMA的诊断仍然具有挑战性，由于没有单一的诊断测试可作为金标准，因此需要使用一套标准。最初的诊断标准于1994年确定并发布，随后在2010年进行了修订，主要关注右心室受累情况。2019年，一个国际专家小组确定了先前诊断标准的局限性。2020年的帕多瓦标准纳入了诊断左心室变异型的特定途径，并强调在心肌瘢痕特征描述中需要使用心脏磁共振成像。这些标准在2023年进一步完善并作为欧洲工作组（TF）标准发布，从而获得了国际认可。在本综述中，通过探究CMA及其诊断的历史，我们分析了从1994年第一版标准到2023年欧洲TF最新标准这20年间所发生的变化和进展，突出了我们对该疾病病理生物学以及形态功能特征认识的演变。最相关的更新之一无疑是引入了“瘢痕形成/致心律失常性心肌病”的概念——这一定义强化了该病理学的主要特征，并强调了与病因无关的多种表型和临床表现。
