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心肌纤维化:70 年后的最新进展

Endomyocardial Fibrosis: an Update After 70 Years.

机构信息

Division of Non-Communicable Diseases, Universidade Eduardo Mondlane, Faculdade de Medicina, Maputo, Mozambique.

Instituto Nacional de Saúde, Marracuene, Mozambique.

出版信息

Curr Cardiol Rep. 2019 Nov 22;21(11):148. doi: 10.1007/s11886-019-1244-3.

Abstract

PURPOSE OF REVIEW

This review aims at highlighting the need to better understand the pathogenesis and natural history of endomyocardial fibrosis when set against its changing endemicity and disease burden, improvements in diagnosis, and new options for clinical management.

RECENT FINDINGS

Progress in imaging diagnostic techniques and availability of new targets for drug and surgical treatment of heart failure are contributing to earlier diagnosis and may lead to improvement in patient survival. Endomyocardial fibrosis was first described in Uganda by Davies more than 70 years ago (1948). Despite its poor prognosis, the etiology of this neglected tropical restrictive cardiomyopathy still remains enigmatic nowadays. Our review reflects on the journey of scientific discovery and construction of the current guiding concepts on this mysterious and fascinating condition, bringing to light the contemporary knowledge acquired over these years. Here we describe novel tools for diagnosis, give an overview of the improvement in clinical management, and finally, suggest research themes that can help improve patient outcomes focusing (whenever possible) on novel players coming into action.

摘要

目的综述

本综述旨在强调需要更好地理解心肌纤维化的发病机制和自然史,同时要考虑到其不断变化的地方性和疾病负担、诊断方面的改进以及临床管理的新选择。

最近的发现

成像诊断技术的进步和心力衰竭药物及手术治疗新靶点的出现,有助于更早地诊断,并可能提高患者的生存率。心肌纤维化是由 Davies 于 70 多年前(1948 年)在乌干达首次描述的。尽管预后不佳,但这种被忽视的热带限制性心肌病的病因至今仍扑朔迷离。本综述回顾了这一神秘而迷人疾病的科学发现之旅和当前指导概念的构建,揭示了多年来获得的当代知识。在这里,我们描述了用于诊断的新工具,概述了临床管理的改善,最后提出了可以帮助改善患者预后的研究主题,同时(只要有可能)关注新出现的治疗靶点。

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