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扩张型心肌病:诊断与风险分层的综合方法

Dilated Cardiomyopathy: A Comprehensive Approach to Diagnosis and Risk Stratification.

作者信息

Ferreira André, Ferreira Vera, Antunes Miguel Marques, Lousinha Ana, Pereira-da-Silva Tiago, Antunes Diana, Cunha Pedro Silva, Oliveira Mário, Ferreira Rui Cruz, Rosa Sílvia Aguiar

机构信息

Cardiology Service, Hospital Santa Marta, Centro Hospitalar Universitário de Lisboa Central, 1169-050 Lisbon, Portugal.

Department of Genetics, Centro Hospitalar Universitário de Lisboa Central, 1169-050 Lisbon, Portugal.

出版信息

Biomedicines. 2023 Mar 9;11(3):834. doi: 10.3390/biomedicines11030834.

Abstract

Dilated cardiomyopathy (DCM) represents one of the most common causes of non-ischemic heart failure, characterised by ventricular dilation alongside systolic dysfunction. Despite advances in therapy, DCM mortality rates remain high, and it is one of the leading causes of heart transplantation. It was recently recognised that many patients present minor structural cardiac abnormalities and express different arrhythmogenic phenotypes before overt heart-failure symptoms. This has raised several diagnostic and management challenges, including the differential diagnosis with other phenotypically similar conditions, the identification of patients at increased risk of malignant arrhythmias, and of those who will have a worse response to medical therapy. Recent developments in complementary diagnostic procedures, namely cardiac magnetic resonance and genetic testing, have shed new light on DCM understanding and management. The present review proposes a comprehensive and systematic approach to evaluating DCM, focusing on an improved diagnostic pathway and a structured stratification of arrhythmic risk that incorporates novel imaging modalities and genetic test results, which are critical for guiding clinical decision-making and improving outcomes.

摘要

扩张型心肌病(DCM)是导致非缺血性心力衰竭的最常见原因之一,其特征为心室扩张伴收缩功能障碍。尽管治疗取得了进展,但DCM的死亡率仍然很高,并且是心脏移植的主要原因之一。最近发现,许多患者在出现明显的心力衰竭症状之前就存在轻微的心脏结构异常,并表现出不同的致心律失常表型。这带来了一些诊断和管理方面的挑战,包括与其他表型相似疾病的鉴别诊断、识别恶性心律失常风险增加的患者以及对药物治疗反应较差的患者。心脏磁共振成像和基因检测等辅助诊断程序的最新进展,为DCM的理解和管理提供了新的思路。本综述提出了一种全面、系统的方法来评估DCM,重点是改进诊断途径和对心律失常风险进行结构化分层,该分层纳入了新型成像模式和基因检测结果,这些对于指导临床决策和改善治疗效果至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d7c6/10044994/cde4f71f3bf6/biomedicines-11-00834-g001.jpg

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