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扩张型心肌病的概念演变。

Evolving concepts in dilated cardiomyopathy.

机构信息

Cardiovascular Department 'Ospedali Riuniti' and University of Trieste, Trieste, Italy.

Centre for Heart Muscle Disease, Institute of Cardiological Sciences, University College London and St. Bartholomew's Hospital, London, UK.

出版信息

Eur J Heart Fail. 2018 Feb;20(2):228-239. doi: 10.1002/ejhf.1103. Epub 2017 Dec 22.

Abstract

Dilated cardiomyopathy (DCM) represents a particular aetiology of systolic heart failure that frequently has a genetic background and usually affects young patients with few co-morbidities. The prognosis of DCM has improved substantially during the last decades due to more accurate aetiological characterization, the red-flag integrated approach to the disease, early diagnosis through systematic familial screening, and the concept of DCM as a dynamic disease requiring constant optimization of medical and non-pharmacological evidence-based treatments. However, some important issues in clinical management remain unresolved, including the role of cardiac magnetic resonance for diagnosis and risk categorization and the interaction between genotype and clinical phenotype, and arrhythmic risk stratification. This review offers a comprehensive survey of these and other emerging issues in the clinical management of DCM, providing where possible practical recommendations.

摘要

扩张型心肌病(DCM)是一种以收缩性心力衰竭为特征的特定病因,常具有遗传背景,且通常影响合并症较少的年轻患者。由于病因学的更准确描述、疾病的综合危险分层方法、通过系统的家族筛查进行早期诊断以及将 DCM 视为需要不断优化基于证据的药物和非药物治疗的动态疾病的概念,DCM 的预后在过去几十年中得到了显著改善。然而,临床管理中仍存在一些悬而未决的重要问题,包括心脏磁共振在诊断和危险分层中的作用,基因型与临床表型的相互作用以及心律失常风险分层。本综述全面探讨了 DCM 临床管理中的这些和其他新出现的问题,并尽可能提供了实用建议。

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