Arrhythmic Risk Stratification and Sudden Cardiac Death Prevention in Duchenne Muscular Dystrophy: A Critical Appraisal.

IF 1.9 4区 医学 Q3 CARDIAC & CARDIOVASCULAR SYSTEMS
Reviews in cardiovascular medicine Pub Date : 2025-03-11 eCollection Date: 2025-03-01 DOI:10.31083/RCM27089
Domenico D'Amario, Alessandra Arcudi, Maria Lucia Narducci, Valeria Novelli, Francesco Canonico, Alessandro Parodi, Gabriele Dell'Era, Marco Di Francesco, Renzo Laborante, Josip Andelo Borovac, Mattia Galli, Eugenio Maria Mercuri, Giuseppe Vergaro, Antonio Dello Russo, Anthea Tonia D'Amico, Antonio Bisignani, Rachele Adorisio, Giulio Pompilio, Giuseppe Patti
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引用次数: 0

Abstract

Duchenne muscular dystrophy (DMD) is a genetic progressive neuromuscular disorder characterized by early-onset proximal muscle weakness and significant long-term pulmonary and cardiac involvement. Due to the early pharmacological treatments and the wider adoption of non-invasive ventilation, life expectancy has significantly increased in recent years, highlighting the relevance of DMD-related cardiomyopathy and fatal arrhythmias, especially in the late stage of the disease. Current guideline-derived evaluation of sudden cardiac death (SCD) in DMD lacks accuracy, leading to inadequate arrhythmic risk stratification and jeopardized SCD prevention strategies. This review aims to outline these critical issues, proposing an integrative approach encompassing manifold tools such as an imaging-derived systematic and comprehensive evaluation (speckle-tracking echocardiography and magnetic resonance imaging), the electrophysiological study, the 3-dimensional electroanatomic mapping, and a multidimensional clinical examination. This approach might lead to more personalized management along with an effective arrhythmia-prevention strategy aiming to balance clinical care goals, patient expectations, and ethical considerations.

杜氏肌营养不良患者心律失常风险分层和心源性猝死预防:一项关键评估。
杜氏肌营养不良症(DMD)是一种遗传性进行性神经肌肉疾病,其特征是早发性近端肌无力和严重的长期肺和心脏累及。由于早期的药物治疗和无创通气的广泛采用,预期寿命近年来显着增加,突出了dmd相关心肌病和致命性心律失常的相关性,特别是在疾病的晚期。目前由指南衍生的对DMD患者心源性猝死(SCD)的评估缺乏准确性,导致心律失常风险分层不充分,并危及SCD预防策略。本综述旨在概述这些关键问题,提出一种综合方法,包括多种工具,如成像衍生的系统和综合评估(斑点跟踪超声心动图和磁共振成像),电生理研究,三维电解剖制图和多维临床检查。这种方法可能会导致更个性化的管理,以及有效的心律失常预防策略,旨在平衡临床护理目标、患者期望和伦理考虑。
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来源期刊
Reviews in cardiovascular medicine
Reviews in cardiovascular medicine 医学-心血管系统
CiteScore
2.70
自引率
3.70%
发文量
377
审稿时长
1 months
期刊介绍: RCM is an international, peer-reviewed, open access journal. RCM publishes research articles, review papers and short communications on cardiovascular medicine as well as research on cardiovascular disease. We aim to provide a forum for publishing papers which explore the pathogenesis and promote the progression of cardiac and vascular diseases. We also seek to establish an interdisciplinary platform, focusing on translational issues, to facilitate the advancement of research, clinical treatment and diagnostic procedures. Heart surgery, cardiovascular imaging, risk factors and various clinical cardiac & vascular research will be considered.
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