[Diagnosis of arrhythmogenic cardiomyopathy: 20 years of progress and innovation].

IF 0.7 Q4 CARDIAC & CARDIOVASCULAR SYSTEMS
Domenico Corrado, Alessandro Zorzi, Barbara Bauce, Ilaria Rigato, Alberto Cipriani, Martina Perazzolo Marra, Kalliopi Pilichou, Cristina Basso, Simone Ungaro, Francesca Graziano
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引用次数: 0

Abstract

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.

[心律失常性心肌病的诊断:20 年的进步与创新]。
心律失常性心肌病(CMA)是一种以非缺血性心室瘢痕和心电不稳定为特征的心脏病。时至今日,CMA 的诊断仍然具有挑战性,需要使用一套标准,因为没有一种诊断测试能代表金标准。第一个诊断标准于 1994 年确定并发布,随后于 2010 年进行了修订,主要侧重于右心室受累。2019 年,一个国际专家小组确定了之前诊断标准的局限性。2020 年的帕多瓦标准包括了诊断左心室变异的具体途径,并强调了使用心脏磁共振成像确定心肌瘢痕特征的必要性。本综述探讨了 CMA 及其诊断的历史,分析了从 1994 年第一版标准到 2023 年最新的欧洲 TF 标准这 20 年间的变化和进展,强调了我们对该疾病的病理生物学和形态功能特征的认识的演变。其中最有意义的更新无疑是引入了 "瘢痕/心律失常性心肌病 "的概念,这一定义增强了病理的主要特征,强调了表型和临床表现的多样性,与病因无关。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Giornale italiano di cardiologia
Giornale italiano di cardiologia CARDIAC & CARDIOVASCULAR SYSTEMS-
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