Integrative Approaches in the Management of Hypertrophic Cardiomyopathy: A Comprehensive Review of Current Therapeutic Modalities.

IF 3.9 3区 工程技术 Q2 BIOCHEMISTRY & MOLECULAR BIOLOGY
Marco Maria Dicorato, Gaetano Citarelli, Francesco Mangini, Rossella Alemanni, Miriam Albanese, Sebastiano Cicco, Cosimo Angelo Greco, Cinzia Forleo, Paolo Basile, Maria Cristina Carella, Marco Matteo Ciccone, Andrea Igoren Guaricci, Ilaria Dentamaro
{"title":"Integrative Approaches in the Management of Hypertrophic Cardiomyopathy: A Comprehensive Review of Current Therapeutic Modalities.","authors":"Marco Maria Dicorato, Gaetano Citarelli, Francesco Mangini, Rossella Alemanni, Miriam Albanese, Sebastiano Cicco, Cosimo Angelo Greco, Cinzia Forleo, Paolo Basile, Maria Cristina Carella, Marco Matteo Ciccone, Andrea Igoren Guaricci, Ilaria Dentamaro","doi":"10.3390/biomedicines13051256","DOIUrl":null,"url":null,"abstract":"<p><p>Hypertrophic cardiomyopathy (HCM) is often associated with left ventricular outflow tract (LVOT) obstruction, which affects a substantial proportion of patients. This obstruction results from a range of anatomical abnormalities involving both the valvular and subvalvular structures. Pharmacological therapies play a pivotal role in the management of LVOT obstruction, with a range of drug classes exhibiting distinct mechanisms of action. Beta-blockers, including atenolol and nadolol, are considered the first-line treatment due to their ability to reduce heart rate and myocardial contractility and enhance diastolic filling. Non-dihydropyridine calcium channel blockers, such as verapamil and diltiazem, are utilized as second-line agents when beta-blockers are ineffective or contraindicated. Disopyramid, a Class 1A antiarrhythmic agent, is employed for patients who do not respond to initial therapeutic interventions and can reduce LVOT gradients. Recent advancements in cardiac myosin modulators, such as Mavacamten and Aficamten, offer targeted therapies by modulating myosin-actin interactions to reduce LVOT gradients and improve symptoms, with promising results from clinical trials. Although gene therapy is still in its nascent stages, it has the potential to address the genetic basis of HCM by employing techniques such as genome editing, gene replacement, and the modulation of signaling pathways. For patients exhibiting severe symptoms or demonstrating unresponsiveness to medical treatment, invasive therapies, such as septal reduction therapy and alcohol septal ablation, are considered. Ultimately, the treatment and prevention of atrial fibrillation and sudden cardiac death are two key points of HCM management in both obstructive and non-obstructive forms. This review aims to provide an overview of current pharmacological and invasive strategies, as well as emerging therapies, in the management of HCM.</p>","PeriodicalId":8937,"journal":{"name":"Biomedicines","volume":"13 5","pages":""},"PeriodicalIF":3.9000,"publicationDate":"2025-05-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12108688/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Biomedicines","FirstCategoryId":"5","ListUrlMain":"https://doi.org/10.3390/biomedicines13051256","RegionNum":3,"RegionCategory":"工程技术","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"BIOCHEMISTRY & MOLECULAR BIOLOGY","Score":null,"Total":0}
引用次数: 0

Abstract

Hypertrophic cardiomyopathy (HCM) is often associated with left ventricular outflow tract (LVOT) obstruction, which affects a substantial proportion of patients. This obstruction results from a range of anatomical abnormalities involving both the valvular and subvalvular structures. Pharmacological therapies play a pivotal role in the management of LVOT obstruction, with a range of drug classes exhibiting distinct mechanisms of action. Beta-blockers, including atenolol and nadolol, are considered the first-line treatment due to their ability to reduce heart rate and myocardial contractility and enhance diastolic filling. Non-dihydropyridine calcium channel blockers, such as verapamil and diltiazem, are utilized as second-line agents when beta-blockers are ineffective or contraindicated. Disopyramid, a Class 1A antiarrhythmic agent, is employed for patients who do not respond to initial therapeutic interventions and can reduce LVOT gradients. Recent advancements in cardiac myosin modulators, such as Mavacamten and Aficamten, offer targeted therapies by modulating myosin-actin interactions to reduce LVOT gradients and improve symptoms, with promising results from clinical trials. Although gene therapy is still in its nascent stages, it has the potential to address the genetic basis of HCM by employing techniques such as genome editing, gene replacement, and the modulation of signaling pathways. For patients exhibiting severe symptoms or demonstrating unresponsiveness to medical treatment, invasive therapies, such as septal reduction therapy and alcohol septal ablation, are considered. Ultimately, the treatment and prevention of atrial fibrillation and sudden cardiac death are two key points of HCM management in both obstructive and non-obstructive forms. This review aims to provide an overview of current pharmacological and invasive strategies, as well as emerging therapies, in the management of HCM.

肥厚性心肌病的综合治疗方法:当前治疗方式的综合综述。
肥厚性心肌病(HCM)常伴有左心室流出道(LVOT)梗阻,影响相当大比例的患者。这种梗阻是由一系列涉及瓣膜和瓣膜下结构的解剖异常引起的。药物治疗在LVOT阻塞的治疗中起着关键作用,有一系列的药物类别表现出不同的作用机制。-受体阻滞剂,包括阿替洛尔和纳多洛尔,被认为是一线治疗,因为它们能够降低心率和心肌收缩力,增强舒张充盈。非二氢吡啶钙通道阻滞剂,如维拉帕米和地尔硫卓,在β受体阻滞剂无效或禁忌时用作二线药物。双金字塔是一种1A类抗心律失常药物,用于对初始治疗干预无反应的患者,可以降低LVOT梯度。心脏肌球蛋白调节剂的最新进展,如马伐camten和Aficamten,通过调节肌球蛋白-肌动蛋白相互作用提供靶向治疗,以降低LVOT梯度并改善症状,临床试验结果令人期待。尽管基因治疗仍处于初级阶段,但通过采用基因组编辑、基因替代和信号通路调节等技术,它有可能解决HCM的遗传基础问题。对于表现出严重症状或对药物治疗无反应的患者,可考虑采用侵入性治疗,如室间隔缩小治疗和酒精室间隔消融。最终,治疗和预防房颤和心源性猝死是HCM管理的两个关键点,无论是梗阻性还是非梗阻性。这篇综述的目的是提供当前的药理学和侵入性策略,以及新兴的治疗HCM的概述。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 求助全文
来源期刊
Biomedicines
Biomedicines Biochemistry, Genetics and Molecular Biology-General Biochemistry,Genetics and Molecular Biology
CiteScore
5.20
自引率
8.50%
发文量
2823
审稿时长
8 weeks
期刊介绍: Biomedicines (ISSN 2227-9059; CODEN: BIOMID) is an international, scientific, open access journal on biomedicines published quarterly online by MDPI.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信