Graziano Francesca, Zorzi Alessandro, Cipriani Alberto, De Lazzari Manuel, Bauce Barbara, Rigato Ilaria, Brunetti Giulia, Pilichou Kalliopi, Basso Cristina, Perazzolo Marra Martina, Corrado Domenico
Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, 35128 Padova, Italy.
Rev Cardiovasc Med. 2022 Oct 10;23(10):335. doi: 10.31083/j.rcm2310335. eCollection 2022 Oct.
Arrhythmogenic cardiomyopathy (ACM) is a rare heart muscle disease characterized by a progressive fibro-fatty myocardial replacement, ventricular arrhythmias, and increased risk of sudden cardiac death. The first diagnostic criteria were proposed by an International Task Force of experts in 1994 and revised in 2010. At that time, ACM was mainly considered a right ventricle disease, with left ventricle involvement only in the late stages. Since 2010, several pathological and clinical studies using cardiac magnetic resonance (CMR) imaging have allowed to understand the phenotypic expression of the disease and to reach the current idea that ACM may affect both ventricles. Indeed, left ventricular involvement may parallel or exceed right ventricular involvement. The main limitations of the 2010 criteria included the poor sensitivity for left ventricular involvement and the lack of inclusion of tissue characterization by CMR. The 2020 International criteria (the Padua criteria) were developed to overcome these shortcomings. The most important innovations are the introduction of a set of criteria for identifying left ventricular variants and the use of CMR for tissue characterization. Moreover, criteria for right ventricular involvement were also updated taking into account new evidence. According to the number of criteria for right and/or left ventricular involvement, the 2020 Padua criteria allows diagnosing three ACM phenotypic variants: right-dominant, biventricular and left-dominant. This review discusses the evolving approach to diagnosis of ACM, from the 1994 International Criteria to the 2020 Padua criteria.
致心律失常性心肌病（ACM）是一种罕见的心肌疾病，其特征为进行性纤维脂肪性心肌替代、室性心律失常以及心脏性猝死风险增加。1994年，一个国际专家特别工作组提出了首批诊断标准，并于2010年进行了修订。当时，ACM主要被认为是一种右心室疾病，仅在晚期累及左心室。自2010年以来，多项使用心脏磁共振（CMR）成像的病理和临床研究有助于了解该疾病的表型表达，并形成了目前ACM可能累及双心室的观点。事实上，左心室受累可能与右心室受累并行或超过右心室受累。2010年标准的主要局限性包括对左心室受累的敏感性较差以及未纳入CMR的组织特征描述。2020年国际标准（帕多瓦标准）的制定就是为了克服这些缺点。最重要的创新之处在于引入了一套识别左心室变异型的标准以及使用CMR进行组织特征描述。此外，还根据新证据更新了右心室受累的标准。根据右心室和/或左心室受累的标准数量，2020年帕多瓦标准可诊断出三种ACM表型变异型：右心室优势型、双心室型和左心室优势型。本综述讨论了从1994年国际标准到2020年帕多瓦标准，ACM诊断方法的演变。
