Contemporary Insights into LMNA Cardiomyopathy.

IF 3.1 3区 医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS
Iswaree D Balakrishnan, Neal K Lakdawala
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引用次数: 0

Abstract

Purpose of review: This review aims to explore how a diagnosis of LMNA-related cardiomyopathy (LMNA-CM) informs clinical management, focusing on the prevention and management of its complications, through practical clinical strategies.

Recent findings: Longitudinal studies have enhanced our understanding of the natural history of LMNA-CM including its arrhythmic and non-arrhythmic complications. A LMNA specific ventricular arrhythmia risk prediction strategy has been integrated into clinical practice guidelines. Although less robust, observational studies are shaping gene-specific strategies for mitigating other complications including atrioventricular block, atrial fibrillation and cardiomyopathy, while novel therapies have been evaluated in clinical trials. LMNA-CM follows an aggressive yet generally stereotyped course. Early recognition of anticipated complications allows for more effective prevention and management in both symptomatic and asymptomatic patients.

综述目的:本综述旨在探讨 LMNA 相关心肌病(LMNA-CM)的诊断如何为临床管理提供依据,重点是通过实用的临床策略预防和管理其并发症:纵向研究加深了我们对 LMNA-CM 自然史的了解,包括其心律失常和非心律失常并发症。一项针对 LMNA 的室性心律失常风险预测策略已被纳入临床实践指南。观察性研究虽然不那么有力,但正在形成减轻其他并发症(包括房室传导阻滞、心房颤动和心肌病)的基因特异性策略,同时在临床试验中对新型疗法进行了评估。LMNA-CM 病程凶险,但一般已定型。对预期并发症的早期识别可以更有效地预防和治疗有症状和无症状的患者。
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来源期刊
Current Cardiology Reports
Current Cardiology Reports CARDIAC & CARDIOVASCULAR SYSTEMS-
CiteScore
6.20
自引率
2.70%
发文量
209
期刊介绍: The aim of this journal is to provide timely perspectives from experts on current advances in cardiovascular medicine. We also seek to provide reviews that highlight the most important recently published papers selected from the wealth of available cardiovascular literature. We accomplish this aim by appointing key authorities in major subject areas across the discipline. Section editors select topics to be reviewed by leading experts who emphasize recent developments and highlight important papers published over the past year. An Editorial Board of internationally diverse members suggests topics of special interest to their country/region and ensures that topics are current and include emerging research. We also provide commentaries from well-known figures in the field.
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