Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Mar 1;10(5):951.
doi: 10.3390/jcm10050951.

Emerging Medical Treatment for Hypertrophic Cardiomyopathy

Affiliations
Review

Emerging Medical Treatment for Hypertrophic Cardiomyopathy

Alessia Argirò et al. J Clin Med. .

Abstract

Hypertrophic cardiomyopathy (HCM) is a common myocardial disease characterized by otherwise unexplained left ventricular hypertrophy. The main cause of disabling symptoms in patients with HCM is left ventricular outflow tract (LVOT) obstruction. This phenomenon is multifactorial, determined both by anatomical and functional abnormalities: myocardial hypercontractility is believed to represent one of its major determinants. The anatomical anomalies are targeted by surgical interventions, whereas attenuating hypercontractility is the objective of old and new drugs including the novel class of allosteric myosin inhibitors. This review summarizes the current treatment modalities and discusses the emerging therapeutical opportunities focusing on the recently developed cardiac myosin ATPase inhibitors Mavacamten and CK-274. Novel surgical and interventional approaches are also discussed.

Keywords: hypertrophic cardiomyopathy; left ventricular outflow tract obstruction; mavacamten; septal reduction therapy.

PubMed Disclaimer

Conflict of interest statement

IO is a consultant for Myokardia and Cytokinetics.

Figures

Figure 1
Figure 1
Structural model of the open on-state and the closed-off state of human beta cardiac myosin. Mavacamten and CK-274 shift the on-off state equilibrium toward the off-folded state, ultimately reducing contractility, in the opposite way Omecamtiv mecarbil stabilizes the open state increasing contractility.
Figure 2
Figure 2
Possible treatment algorithm for obstructive Hypertrophic Cardiomyopathy therapy. HCM: Hypertrophic Cardiomyopathy. * Mavacamten has been submitted for Food and Drugs administration approval. See the text for the flow chart description.

References

    1. Maron B.J., Gardin J.M., Flack J.M., Gidding S.S., Kurosaki T.T., Bild D.E. Prevalence of Hypertrophic Cardiomyopathy in a General Population of Young Adults: Echocardiographic Analysis of 4111 Subjects in the CARDIA Study. Circulation. 1995;92:785–789. doi: 10.1161/01.CIR.92.4.785. - DOI - PubMed
    1. Maron B.J., Maron M.S., Semsarian C. Genetics of Hypertrophic Cardiomyopathy After 20 Years. J. Am. Coll. Cardiol. 2012;60:705–715. doi: 10.1016/j.jacc.2012.02.068. - DOI - PubMed
    1. Olivotto I., Cecchi F., Poggesi C., Yacoub M.H. Developmental origins of hypertrophic cardiomyopathy phenotypes: A unifying hypothesis. Nat. Rev. Cardiol. 2009;6:317–321. doi: 10.1038/nrcardio.2009.9. - DOI - PubMed
    1. Maron B.J., Rowin E.J., Udelson J.E., Maron M.S. Clinical Spectrum and Management of Heart Failure in Hypertrophic Cardiomyopathy. JACC Heart Fail. 2018;6:353–363. doi: 10.1016/j.jchf.2017.09.011. - DOI - PubMed
    1. Maron M.S., Olivotto I., Betocchi S., Casey S.A., Lesser J.R., Losi M.A., Cecchi F., Maron B.J. Effect of Left Ventricular Outflow Tract Obstruction on Clinical Outcome in Hypertrophic Cardiomyopathy. N. Engl. J. Med. 2003;348:295–303. doi: 10.1056/NEJMoa021332. - DOI - PubMed