Pathophysiology and Treatment of Hypertrophic Cardiomyopathy: New Perspectives

Overview

Journal Curr Heart Fail Rep

Publisher Current Science

Date 2021 Jun 20

PMID 34148184

Citations 15

Authors

Mattia Zampieri

Martina Berteotti

Cecilia Ferrantini

Luigi Tassetti

Martina Gabriele

Benedetta Tomberli

Gabriele Castelli

Francesco Cappelli

Pierluigi Stefano

Niccolo Marchionni

Raffaele Coppini

Iacopo Olivotto

Affiliations

Soon will be listed here.

Abstract

Purpose Of Review: We provide a state of the art of therapeutic options in hypertrophic cardiomyopathy (HCM), focusing on recent advances in our understanding of the pathophysiology of sarcomeric disease.

Recent Findings: A wealth of novel information regarding the molecular mechanisms associated with the clinical phenotype and natural history of HCM have been developed over the last two decades. Such advances have only recently led to a number of controlled randomized studies, often limited in size and fortune. Recently, however, the allosteric inhibitors of cardiac myosin adenosine triphosphatase, countering the main pathophysiological abnormality associated with HCM-causing mutations, i.e. hypercontractility, have opened new management perspectives. Mavacamten is the first drug specifically developed for HCM used in a successful phase 3 trial, with the promise to reach symptomatic obstructive patients in the near future. In addition, the fine characterization of cardiomyocyte electrophysiological remodelling has recently highlighted relevant therapeutic targets. Current therapies for HCM focus on late disease manifestations without addressing the intrinsic pathological mechanisms. However, novel evidence-based approaches have opened the way for agents targeting HCM molecular substrates. The impact of these targeted interventions will hopefully alter the natural history of the disease in the near future.

Citing Articles

Hypertrophic Cardiomyopathy with Special Focus on Mavacamten and Its Future in Cardiology.

Mlynarska E, Radzioch E, Dabek B, Leszto K, Witkowska A, Czarnik W Biomedicines. 2025; 12(12.

PMID: 39767581 PMC: 11727519. DOI: 10.3390/biomedicines12122675.

Hypertrophic Cardiomyopathy as a Form of Heart Failure with Preserved Ejection Fraction: Diagnosis, Drugs, and Procedures.

Banthiya S, Check L, Atkins J US Cardiol. 2024; 18:e17.

PMID: 39508003 PMC: 11539043. DOI: 10.15420/usc.2023.21.

Efficacy and safety of mavacamten for the treatment of hypertrophic cardiomyopathy: an updated systematic review and meta-analysis of randomized controlled trials.

Almansouri N, Nadeem Bukhari S, Qureshi M, Idrees M, Riaz C, Asghar A Ann Med Surg (Lond). 2024; 86(10):6097-6104.

PMID: 39359828 PMC: 11444588. DOI: 10.1097/MS9.0000000000002466.

An evidence review and gap analysis for obstructive hypertrophic cardiomyopathy.

Butzner M, Aronitz E, Cameron H, Tantakoun K, Shreay S, Drudge C BMC Cardiovasc Disord. 2024; 24(1):416.

PMID: 39127628 PMC: 11316357. DOI: 10.1186/s12872-024-04084-7.

Hypertrophic Cardiomyopathy: From Medical Treatment to Advanced Heart Failure Therapies.

Mazur M, Braksator W, Popjes E Curr Cardiol Rep. 2024; 26(9):985-994.

PMID: 38990491 DOI: 10.1007/s11886-024-02095-6.

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