[指南更新:基于表型的心肌病治疗]。

Deutsche medizinische Wochenschrift (1946) Pub Date : 2025-03-01 Epub Date: 2025-02-21 DOI:10.1055/a-2271-4980
Ali Amr, Norbert Frey, Benjamin Meder
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引用次数: 0

摘要

2023 ESC心肌病指南为心肌病的诊断和管理提供了一个全面的框架。指南建立在一个细致入微的分类系统上,引入了整合遗传和非遗传病因的表型描述。值得注意的是,该指南重新定义了心肌病,如非扩张型左室心肌病,强调了详细的心肌组织特征和先进的成像技术,如心脏磁共振,以提高诊断和治疗。此外,强调了基因检测的作用,包括家庭筛查和个体化风险分层,以预防心源性猝死。该指南强调以患者为中心的多学科方法,确保从儿科到成人护理的所有生命阶段的个性化护理。关键的更新包括新的治疗选择,如肥厚性心肌病的肌球蛋白抑制剂。该指南还强调了将短暂综合征(如Takotsubo综合征)与慢性心肌病区分开来的重要性,建议仔细评估心律失常和表型特征,以避免错误分类。这种改进的方法旨在通过准确的诊断、基因评估和终身管理来优化临床结果。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Guideline update: phenotype-based management of cardiomyopathy].

The 2023 ESC Cardiomyopathy Guidelines offer a comprehensive framework for diagnosing and managing cardiomyopathies. Building on a nuanced classification system, the guidelines introduce phenotypic descriptions that integrate genetic and non-genetic etiologies. Notably, the guidelines redefine cardiomyopathies, such as non-dilated left ventricular cardiomyopathy, emphasizing detailed myocardial tissue characterization and advanced imaging techniques like cardiac magnetic resonance to enhance diagnosis and treatment. Additionally, the role of genetic testing is highlighted, including family screening and personalized risk stratification for sudden cardiac death prevention. The guidelines stress a patient-centered, multidisciplinary approach, ensuring individualized care across all life stages, from pediatric to adult care. Key updates include new therapeutic options, such as myosin inhibitors for hypertrophic cardiomyopathy. The guidelines also underscore the importance of distinguishing transient syndromes, such as Takotsubo syndrome, from chronic cardiomyopathies, recommending careful assessment of arrhythmias and phenotypic traits to avoid misclassification. This refined approach aims to optimize clinical outcomes through accurate diagnosis, genetic evaluation, and a focus on lifelong management.

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