Bjerregaard Caroline Løkke, Biering-Sørensen Tor, Skaarup Kristoffer Grundtvig, Sengeløv Morten, Lassen Mats Christian Højbjerg, Johansen Niklas Dyrby, Olsen Flemming Javier
Department of Cardiology, Copenhagen University Hospital-Herlev and Gentofte, 2900 Hellerup, Denmark.
Center for Translational Cardiology and Pragmatic Randomized Trials, Department of Biomedical Sciences, Faculty of Health and Medical Sciences, University of Copenhagen, 2200 Copenhagen, Denmark.
J Clin Med. 2024 Jan 26;13(3):717. doi: 10.3390/jcm13030717.
Arrhythmogenic right ventricular cardiomyopathy is an inherited cardiomyopathy, characterized by abnormal cell adhesions, disrupted intercellular signaling, and fibrofatty replacement of the myocardium. These changes serve as a substrate for ventricular arrhythmias, placing patients at risk of sudden cardiac death, even in the early stages of the disease. Current echocardiographic criteria for diagnosing arrhythmogenic right ventricular cardiomyopathy lack sensitivity, but novel markers of cardiac deformation are not subject to the same technical limitations as current guideline-recommended measures. Measuring cardiac deformation using speckle tracking allows for meticulous quantification of global systolic function, regional function, and dyssynchronous contraction. Consequently, speckle tracking to quantify myocardial strain could potentially be useful in the diagnostic process for the determination of disease progression and to assist risk stratification for ventricular arrhythmias and sudden cardiac death. This narrative review provides an overview of the potential use of different myocardial right ventricular strain measures for characterizing right ventricular dysfunction in arrhythmogenic right ventricular cardiomyopathy and its utility in assessing the risk of ventricular arrhythmias.
致心律失常性右室心肌病是一种遗传性心肌病，其特征为细胞黏附异常、细胞间信号传导中断以及心肌纤维脂肪替代。这些改变成为室性心律失常的基础，使患者即使在疾病早期也面临心源性猝死风险。目前用于诊断致心律失常性右室心肌病的超声心动图标准缺乏敏感性，但新型心脏变形标志物不存在当前指南推荐措施所具有的相同技术局限性。使用斑点追踪测量心脏变形可对整体收缩功能、局部功能及不同步收缩进行精确量化。因此，通过斑点追踪量化心肌应变在疾病进展判定的诊断过程中以及协助室性心律失常和心源性猝死风险分层方面可能具有实用价值。本叙述性综述概述了不同右室心肌应变测量方法在致心律失常性右室心肌病中用于表征右室功能障碍的潜在用途及其在评估室性心律失常风险方面的效用。
