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, Via Giustiniani 2, 35128 Padova, Italy.
J Clin Med. 2022 Jan 5;11(1):279. doi: 10.3390/jcm11010279.
Arrhythmogenic Cardiomyopathy (ACM) is a heredo-familial cardiac disease characterized by fibro-fatty myocardial replacement and increased risk of sudden cardiac death. The diagnosis of ACM can be challenging due to the lack of a single gold-standard test: for this reason, it is required to satisfy a combination of multiple criteria from different categories including ventricular morpho-functional abnormalities, repolarization and depolarization ECG changes, ventricular arrhythmias, tissue characterization findings and positive family history/molecular genetics. The first diagnostic criteria were published by an International Task Force (ITF) of experts in 1994 and revised in 2010 with the aim to increase sensitivity for early diagnosis. Limitations of the 2010 ITF criteria include the absence of specific criteria for left ventricle (LV) involvement and the limited role of cardiac magnetic resonance (CMR) as the use of the late gadolinium enhancement technique for tissue characterization was not considered. In 2020, new diagnostic criteria ("the Padua criteria") were proposed. The traditional organization in six categories of major/minor criteria was maintained. The criteria for identifying the right ventricular involvement were modified and a specific set of criteria for identifying LV involvement was created. Depending on the combination of criteria for right and LV involvement, a diagnosis of classic (right dominant) ACM, biventricular ACM or left-dominant ACM is then made. The article reviews the rationale of the Padua criteria, summarizes the main modifications compared to the previous 2010 ITF criteria and provides three examples of the application of the Padua criteria in clinical practice.
致心律失常性心肌病(ACM)是一种遗传性家族性心脏病,其特征为心肌纤维脂肪替代以及心脏性猝死风险增加。由于缺乏单一的金标准检测方法,ACM的诊断具有挑战性:因此,需要满足来自不同类别的多个标准的组合,包括心室形态功能异常、心电图复极和去极变化、室性心律失常、组织特征发现以及阳性家族史/分子遗传学。首批诊断标准由一个国际专家工作组(ITF)于1994年发布,并于2010年进行了修订,目的是提高早期诊断的敏感性。2010年ITF标准的局限性包括缺乏左心室(LV)受累的具体标准,以及心脏磁共振(CMR)的作用有限,因为未考虑使用钆延迟增强技术进行组织特征分析。2020年,提出了新的诊断标准(“帕多瓦标准”)。保留了主要/次要标准分为六类的传统分类方式。确定右心室受累的标准进行了修改,并制定了一套确定LV受累的具体标准。根据右心室和LV受累标准的组合,然后做出经典型(右心室为主)ACM、双心室ACM或左心室为主型ACM的诊断。本文回顾了帕多瓦标准的基本原理,总结了与之前2010年ITF标准相比的主要修改内容,并提供了帕多瓦标准在临床实践中的三个应用实例。