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Targeted Medical Therapies for Hypertrophic Cardiomyopathy

  • Myocardial Disease (A Abbate and G Sinagra, Section Editors)
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Abstract

Purpose of Review

The management of hypertrophic cardiomyopathy (HCM) has changed considerably over the years, although molecular therapies targeting core mechanisms of the disease are still lacking. This review provides an overview of the contemporary medical approach to patients with HCM, and of promising novel developments hopefully soon to enter the clinical arena.

Recent Findings

Our perception of therapeutic targets for medical therapy in HCM is rapidly evolving. Novel approaches include myocardial metabolic modulation, late sodium current inhibition, and allosteric myosin inhibition, actively pursued to reduce and hopefully prevent the development of severe HCM phenotypes, improve symptom control, and preserve patients from disease-related complications.

Summary

Clinical management of patients with HCM should be guided by in-depth knowledge of the complex mechanisms at the energetic, metabolic, and electrophysiologic level. Until new experimental therapies become available, tailored management of modifiable disease manifestations should be pursued, including lifestyle counseling and prevention of comorbidities.

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Abbreviations

AF:

Atrial fibrillation

ASA:

Alcohol septal ablation

CMR:

Cardiac magnetic resonance

CPET:

Cardiopulmonary exercise test

HCM:

Hypertrophic cardiomyopathy

HOCM:

Hypertrophic obstructive cardiomyopathy

HF:

Heart failure

LGE:

Late gadolinium enhancement

LVEF:

Left ventricular ejection fraction

LVOT:

Left ventricular outflow tract

SAM:

Systolic anterior motion

SCD:

Sudden cardiac death

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Acknowledgments

This work was supported by the European Union’s Horizon 2020 Research and Innovation Programme under Grant Agreement no. 777204: “SILICOFCM - In Silico trials for drug tracing the effects of sarcomeric protein mutations leading to familial cardiomyopathy”; by the Italian Ministry of Health “Left ventricular hypertrophy in aortic valve disease and hypertrophic cardiomyopathy: genetic basis, biophysical correlates, and viral therapy models” (RF-2013-02356787), and by the Ente Cassa di Risparmio di Firenze (bando 2016) “juvenile sudden cardiac death: just know and treat.”

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Authors and Affiliations

  1. Cardiomyopathy Unit, Careggi University Hospital, Florence, Italy

    Carlo Fumagalli, Maria Grazia De Gregorio, Alessia Tomberli, Chiara Chiriatti, Alberto Marchi & Iacopo Olivotto

  2. Cardiovascular and Thoracic Department, Verona University Hospital, Verona, Italy

    Mattia Zampieri

  3. Department of Cardiovascular Sciences, Catholic University of the Sacred Heart, Rome, Italy

    Elisa Fedele

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  1. Carlo Fumagalli
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Correspondence to Carlo Fumagalli.

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Carlo Fumagalli, Maria Grazia De Gregorio, Mattia Zampieri, Elisa Fedele, Alessia Tomberli, Chiara Chiriatti, Alberto Marchi, and Iacopo Olivotto declare that they have no conflict of interest.

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Fumagalli, C., De Gregorio, M.G., Zampieri, M. et al. Targeted Medical Therapies for Hypertrophic Cardiomyopathy. Curr Cardiol Rep 22, 10 (2020). https://doi.org/10.1007/s11886-020-1258-x

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