2023年ESC新版心肌病管理指南:心脏病专家决策的指导路径

IF 1.7 4区 医学 Q3 CARDIAC & CARDIOVASCULAR SYSTEMS
M. Grasso, Davide Bondavalli, Viviana Vilardo, Claudia Cavaliere, Ilaria Gatti, Alessandro Di Toro, Lorenzo Giuliani, Mario Urtis, Michela Ferrari, B. Cattadori, A. Serio, Carlo Pellegrini, E. Arbustini
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引用次数: 0

摘要

在《ESC 2023 指南》中,心肌病被保守地定义为 "在没有冠状动脉疾病、高血压、瓣膜疾病和足以导致观察到的心肌异常的先天性心脏病的情况下,心肌结构和功能异常的心肌疾病"。它们在形态和功能上被分为肥厚型、扩张型、限制型和心律失常型右室心肌病,并增加了左室非扩张型心肌病,描述了尽管心脏成像或组织分析显示存在心肌疾病,但不符合标准疾病定义的中间表型。新的ESC指南提供了 "心肌病诊断方法指南,强调了一般评估和管理问题,并为读者提供了建议的相关证据基础"。文件中包含的建议和意见为建立针对特定心肌病(表型和病因)的治疗路径和确定治疗适应症(包括可能的靶向疗法)提供了工具。这对临床心脏病学和遗传学都有影响,临床心脏病学可通过指南协助制定特定疾病的治疗路径,而遗传学则可在临床和检测中进行深入的表型分析,并参与多学科评估,从而为验证变异体的致病性提供独特的工具。心内膜活检的作用仍未得到充分发挥,而且仅限于特定形式的限制性心肌病、心肌炎和淀粉样变性。需要进行新的研究和开发,以弥补科学与临床之间的差距。最后,向生物信息学、生物工程学、数学和物理学等学科的开放将支持临床心脏病专家对新型人工智能辅助资源进行最佳管理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
The new 2023 ESC guidelines for the management of cardiomyopathies: a guiding path for cardiologist decisions
In the ESC 2023 guidelines, cardiomyopathies are conservatively defined as ‘myocardial disorders in which the heart muscle is structurally and functionally abnormal, in the absence of coronary artery disease, hypertension, valvular disease, and congenital heart disease sufficient to cause the observed myocardial abnormality’. They are morpho-functionally classified as hypertrophic, dilated, restrictive, and arrhythmogenic right ventricular cardiomyopathy with the addition of the left ventricular non-dilated cardiomyopathy that describes intermediate phenotypes not fulfilling standard disease definitions despite the presence of myocardial disease on cardiac imaging or tissue analysis. The new ESC guidelines provide ‘a guide to the diagnostic approach to cardiomyopathies, highlight general evaluation and management issues, and signpost the reader to the relevant evidence base for the recommendations’. The recommendations and suggestions included in the document provide the tools to build up pathways tailored to specific cardiomyopathy (phenotype and cause) and define therapeutic indications, including target therapies where possible. The impact is on clinical cardiology, where disease-specific care paths can be assisted by the guidelines, and on genetics, both clinics and testing, where deep phenotyping and participated multi-disciplinary evaluation provide a unique tool for validating the pathogenicity of variants. The role of endomyocardial biopsy remains underexploited and confined to particular forms of restrictive cardiomyopathy, myocarditis, and amyloidosis. New research and development will be needed to cover the gaps between science and clinics. Finally, the opening up to disciplines such as bioinformatics, bioengineering, mathematics, and physics will support clinical cardiologists in the best governance of the novel artificial intelligence–assisted resources.
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来源期刊
European Heart Journal Supplements
European Heart Journal Supplements 医学-心血管系统
CiteScore
3.00
自引率
0.00%
发文量
575
审稿时长
12 months
期刊介绍: The European Heart Journal Supplements (EHJs) is a long standing member of the ESC Journal Family that serves as a publication medium for supplemental issues of the flagship European Heart Journal. Traditionally EHJs published a broad range of articles from symposia to special issues on specific topics of interest. The Editor-in-Chief, Professor Roberto Ferrari, together with his team of eminent Associate Editors: Professor Francisco Fernández-Avilés, Professors Jeroen Bax, Michael Böhm, Frank Ruschitzka, and Thomas Lüscher from the European Heart Journal, has implemented a change of focus for the journal. This entirely refreshed version of the European Heart Journal Supplements now bears the subtitle the Heart of the Matter to give recognition to the focus the journal now has. The EHJs – the Heart of the Matter intends to offer a dedicated, scientific space for the ESC, Institutions, National and Affiliate Societies, Associations, Working Groups and Councils to disseminate their important successes globally.
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