Department of Cardiology II: Electrophysiology, University Hospital Münster, Albert-Schweitzer-Campus 1, 48149, Münster, Germany.
Herzschrittmacherther Elektrophysiol. 2023 Dec;34(4):311-323. doi: 10.1007/s00399-023-00975-y. Epub 2023 Nov 16.
Electrocardiographic findings and arrhythmias are common in cardiomyopathies. Both may be an early indication of a specific diagnosis or may occur due to myocardial fibrosis and/or reduced contractility. Brady- and tachyarrhythmias significantly contribute to increased morbidity and mortality in patients with cardiomyopathies. Antiarrhythmic therapy including risk stratification is often challenging and plays a major role for these patients. Thus, an "electrophysiological" perspective on guidelines on cardiomyopathies may be warranted. As the European Society of Cardiology (ESC) has recently published a new guideline for the management of cardiomyopathies, this overview aims to present key messages of these guidelines. Innovations include a new phenotype-based classification system with emphasis on a multimodal imaging approach for diagnosis and risk stratification. The guideline includes detailed chapters on dilated and hypertrophic cardiomyopathy and their phenocopies, arrhythmogenic right ventricular cardiomyopathy, and restrictive cardiomyopathy as well as syndromic and metabolic cardiomyopathies. Patient pathways guide clinicians from the initial presentation to diagnosis. The role of cardiovascular magnetic resonance imaging and genetic testing during diagnostic work-up is stressed. Concepts of rhythm and rate control for atrial fibrillation have led to new recommendations, and the role of defibrillator therapy in primary prevention is discussed in detail. Whilst providing general guidelines for management, the primary objective of the guideline is to ascertain the disease etiology and disease-specific, individualized management.
心电图表现和心律失常在心肌病中很常见。这些可能是特定诊断的早期迹象,也可能由于心肌纤维化和/或收缩力降低而发生。缓慢性和快速性心律失常显著增加了心肌病患者的发病率和死亡率。抗心律失常治疗包括风险分层,通常具有挑战性,对这些患者起着重要作用。因此,从电生理的角度来看,对心肌病指南进行审查可能是合理的。由于欧洲心脏病学会 (ESC) 最近发布了一份关于心肌病管理的新指南,本综述旨在介绍这些指南的关键信息。创新之处包括一种新的基于表型的分类系统,强调了一种多模式成像方法用于诊断和风险分层。该指南详细介绍了扩张型和肥厚型心肌病及其表型、致心律失常性右心室心肌病和限制性心肌病以及综合征和代谢性心肌病。患者路径指导临床医生从初始表现到诊断。强调了心血管磁共振成像和基因检测在诊断中的作用。房颤的节律和心率控制概念导致了新的建议,并且详细讨论了除颤器治疗在一级预防中的作用。虽然为管理提供了一般指南,但该指南的主要目的是确定疾病病因和特定于疾病的个体化管理。