Recent Therapy and Clinical Approaches for Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is a genetic cardiac condition characterized by abnormal thickening of the myocardium, often due to mutations in sarcomere protein genes. This leads to complications like arrhythmias, left ventricular outflow tract obstruction (LVOT), and sudden cardiac death (SCD). Recent advancements have highlighted the importance of early diagnosis using echocardiography, genetic testing, and advanced imaging. Among the therapeutic innovations, Mavacamten, an allosteric cardiac myosin ATPase inhibitor, offers a novel approach by reducing myocardial hypercontractility and improving LVOT gradients. Clinical trials like EXPLORER-HCM have shown significant improvement in cardiac function and patient quality of life with Mavacamten. Furthermore, the role of implantable cardioverter-defibrillators (ICDs) in preventing SCD has been substantiated, especially with the advent of subcutaneous ICDs, which eliminate intravascular lead-related risks. The integration of pharmacological advances like Mavacamten with ICDs provides a synergistic strategy to manage HCM effectively. Despite these advancements, further research is warranted to explore long-term outcomes and optimize therapeutic protocols, particularly in genetically predisposed populations. This review underscores the need for interdisciplinary approaches to enhance HCM treatment and improve patient prognosis.

Publication Details

Journal
Zenodo (CERN European Organization for Nuclear Research)
Published
2025-03-10
DOI
https://doi.org/10.5281/zenodo.14998472
Primary Topic
Cardiomyopathy and Myosin Studies
Type
article
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article

Recent Therapy and Clinical Approaches for Hypertrophic Cardiomyopathy

Zenodo (CERN European Organization for Nuclear Research)
Cardiomyopathy and Myosin Studies
article

Recent Therapy and Clinical Approaches for Hypertrophic Cardiomyopathy

article en

Abstract

Hypertrophic cardiomyopathy (HCM) is a genetic cardiac condition characterized by abnormal thickening of the myocardium, often due to mutations in sarcomere protein genes. This leads to complications like arrhythmias, left ventricular outflow tract obstruction (LVOT), and sudden cardiac death (SCD). Recent advancements have highlighted the importance of early diagnosis using echocardiography, genetic testing, and advanced imaging. Among the therapeutic innovations, Mavacamten, an allosteric cardiac myosin ATPase inhibitor, offers a novel approach by reducing myocardial hypercontractility and improving LVOT gradients. Clinical trials like EXPLORER-HCM have shown significant improvement in cardiac function and patient quality of life with Mavacamten. Furthermore, the role of implantable cardioverter-defibrillators (ICDs) in preventing SCD has been substantiated, especially with the advent of subcutaneous ICDs, which eliminate intravascular lead-related risks. The integration of pharmacological advances like Mavacamten with ICDs provides a synergistic strategy to manage HCM effectively. Despite these advancements, further research is warranted to explore long-term outcomes and optimize therapeutic protocols, particularly in genetically predisposed populations. This review underscores the need for interdisciplinary approaches to enhance HCM treatment and improve patient prognosis.

Zenodo (CERN European Organization for Nuclear Research)
Openalex Percentile: Top 41%
Cardiomyopathy and Myosin Studies
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