MAVACAMTEN IN THE TREATMENT OF HYPERTROPHIC CARDIOMYOPATHY

Authors

DOI:

https://doi.org/10.47820/recima21.v4i9.4044

Keywords:

Left ventricle. Hypertrophy. Medicine.

Abstract

Introduction: Hypertrophic cardiomyopathy (HCM) is one of the most common genetic heart diseases, affecting between 0.16% and 0.29% of the general adult population. It is a genetic disease of the myocardium and is characterized by marked myocardial hypertrophy that cannot be explained by the pressure load or the presence of myocytic disorder. Objectives: to analyze the effectiveness of mavacamten in the treatment of HCM according to high-quality studies already available in the literature. Materials and methods: This is an integrative review, in which the guiding question was “Is mavacamten an effective drug in the treatment of obstructive hypertrophic cardiomyopathy?”. The search for articles was performed in the PubMed database using the terms “mavacamten” and “hypertrophic obstructive cardiomyopathy”, combined with Boolean operators. Results and Discussion: Treatment with mavacamten improved exercise capacity, left ventricular outflow tract obstruction, New York Heart Association functional class, and health status in patients with obstructive hypertrophic cardiomyopathy. Mavacamten was associated with favorable changes in cardiac structure and function over 30 weeks of therapy, including improvement in echocardiographic markers of left ventricular filling pressures, left ventricular outflow tract gradients, and systolic forward motion. Conclusion: Mavacamten significantly improved measures of left ventricular diastolic function and systolic anterior motion, in addition, mavacamten significantly improved quality of life among patients with symptomatic obstructive hypertrophic cardiomyopathy.

Downloads

Download data is not yet available.

Author Biographies

Caique Pereira de Paiva

Estudante de Medicina, atualmente acadêmico do 6º período. Centro Universitário Claretiano - Rio Claro.

Ana Clara Godinho de Freitas

Estudante de Medicina, atualmente acadêmico do 6º período. Centro Universitário Claretiano - Rio Claro.

Carla Lana Germinari Souza

Estudante de Medicina, atualmente acadêmica do 6º período. Centro Universitário Claretiano - Rio Claro.

Felipe Tochihide Iamaguti

Estudante de medicina, atualmente acadêmico do 9º período. Centro Universitário Claretiano - Rio Claro.

Genon Henrique Costa Pinto

Estudante de Medicina, atualmente acadêmico do 4º período. Centro Universitário Claretiano - Rio Claro.

Júlia Peruzzi Iamaguti

Estudante de Medicina, atualmente acadêmica do 6º período. Centro Universitário Claretiano - Rio Claro.

Marina Romaquela Rodrigues

Estudante de Medicina, atualmente acadêmico do 6º período. Centro Universitário Claretiano - Rio Claro.

Tagstan Ribeiro Moioli

Centro Universitário Claretiano - Rio Claro.

Taís Barbosa Honorato

Estudante de Medicina, atualmente acadêmica do 6º período. Centro Universitário Claretiano - Rio Claro.

Carollayne Mendonça Rocha

Universidade José do Rosário Vellano - UNIFENAS.

References

Anderson, Robert L., et al. "Deciphering the super relaxed state of human β-cardiac myosin and the mode of action of mavacamten from myosin molecules to muscle fibers." Proceedings of the National Academy of Sciences 115.35 (2018): E8143-E8152.

Batzner A, Schäfers HJ, Borisov KV, Seggewiß H. Hypertrophic Obstructive Cardiomyopathy. Dtsch Arztebl Int. 2019 Jan 25;116(4):47-53. doi: 10.3238/arztebl.2019.0047. PMID: 30855006; PMCID: PMC6415619.

Desai N, Xie J, Wang Y, Sutton MB, Whang J, Fine JT, Garrison LP Jr. Projecting the Long-term Clinical Value of Mavacamten for the Treatment of Obstructive Hypertrophic Cardiomyopathy in the United States: An Assessment of Net Health Benefit. Clin Ther. 2022 Jan;44(1):52-66.e2. doi: 10.1016/j.clinthera.2021.11.006. Epub 2021 Dec 12. PMID: 34911641.

Dong T, Alencherry B, Ospina S, Desai MY. Review of Mavacamten for Obstructive Hypertrophic Cardiomyopathy and Future Directions. Drug Des Devel Ther. 2023 Apr 8;17:1097-1106. doi: 10.2147/DDDT.S368590. PMID: 37064432; PMCID: PMC10094472.

Edelberg JM, Sehnert AJ, Mealiffe ME, et al. The impact of mavacamten on the pathophysiology of hypertrophic cardiomyopathy: a narrative review. Am J Cardiovasc Drugs. 2022 doi: 10.1007/s40256-022-00532-x.

Ercole, F. F., Melo, L. S. D., & Alcoforado, C. L. G. C. (2014). Revisão integrativa versus revisão sistemática. Reme: Revista Mineira de Enfermagem, 18(1), 09-11.

Hegde SM, Lester SJ, Solomon SD, Michels M, Elliott PM, Nagueh SF, Choudhury L, Zemanek D, Zwas DR, Jacoby D, Wang A, Ho CY, Li W, Sehnert AJ, Olivotto I, Abraham TP. Effect of Mavacamten on Echocardiographic Features in Symptomatic Patients With Obstructive Hypertrophic Cardiomyopathy. J Am Coll Cardiol. 2021 Dec 21;78(25):2518-2532. doi: 10.1016/j.jacc.2021.09.1381. PMID: 34915982.

Heitner SB, Jacoby D, Lester SJ, Owens A, Wang A, Zhang D, Lambing J, Lee J, Semigran M, Sehnert AJ. Mavacamten Treatment for Obstructive Hypertrophic Cardiomyopathy: A Clinical Trial. Ann Intern Med. 2019 Jun 4;170(11):741-748. doi: 10.7326/M18-3016. Epub 2019 Apr 30. PMID: 31035291.

Ho CY, Olivotto I, Jacoby D, et al. Study design and rationale of EXPLORER-HCM: evaluation of mavacamten in adults with symptomatic obstructive hypertrophic cardiomyopathy. Circ Heart Fail. 2020;13(6):e006853. doi: 10.1161/CIRCHEARTFAILURE.120.006853.

Koljaja-Batzner A, Pfeiffer B, Seggewiss H. Die hypertrophe Kardiomyopathie - häufig und nicht erkannt. Internistische Praxis. 2018;59:187–201.

Marian AJ, Braunwald E. Hypertrophic cardiomyopathy: genetics, pathogenesis, clinical manifestations, diagnosis, and therapy. Circ Res. 2017;121(7):749–770. doi: 10.1161/CIRCRESAHA.117.311059.

Maron BJ. Clinical course and management of hypertrophic cardiomyopathy. N Engl J Med. 2018;379(20):1977.

Masri A, Olivotto I. Cardiac Myosin Inhibitors as a Novel Treatment Option for Obstructive Hypertrophic Cardiomyopathy: Addressing the Core of the Matter. J Am Heart Assoc. 2022 May 3;11(9):e024656. doi: 10.1161/JAHA.121.024656. Epub 2022 May 3. PMID: 35502770; PMCID: PMC9238628.

Mendes, K. D. S., Silveira, R. C. D. C. P., & Galvão, C. M. (2008). Revisão integrativa: método de pesquisa para a incorporação de evidências na saúde e na enfermagem. Texto & contexto-enfermagem, 17, 758-764.

MyoKardia, Inc., a wholly owned subsidiary of Bristol Myers Squibb. CAMZYOSTM (mavacamten): US prescribing information. 2022.

Nishimura RA, Seggewiss H, Schaff HV. Hypertrophic obstructive cardiomyopathy: surgical myectomy and septal ablation. Circ Res. 2017;121:771–783.

Olivotto I, Oreziak A, Barriales-Villa R, Abraham TP, Masri A, Garcia-Pavia P, Saberi S, Lakdawala NK, Wheeler MT, Owens A, Kubanek M, Wojakowski W, Jensen MK, Gimeno-Blanes J, Afshar K, Myers J, Hegde SM, Solomon SD, Sehnert AJ, Zhang D, Li W, Bhattacharya M, Edelberg JM, Waldman CB, Lester SJ, Wang A, Ho CY, Jacoby D; EXPLORER-HCM study investigators. Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HCM): a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet. 2020 Sep 12;396(10253):759-769. doi: 10.1016/S0140-6736(20)31792-X. Epub 2020 Aug 29. Erratum in: Lancet. 2020 Sep 12;396(10253):758. PMID: 32871100.

Papadakis M, Basu J, Sharma S. Mavacamten: treatment aspirations in hypertrophic cardiomyopathy. Lancet. 2020;396(10253):736–737. doi: 10.1016/S0140-6736(20)31793-1.

Sousa, Luís & Marques-Vieira, Cristina & Severino, Sandy & Antunes, Vanessa. (2017). Metodologia de Revisão Integrativa da Literatura em Enfermagem. Revista Investigação Enfermagem. 2. 17-26.

Xie J, Wang Y, Xu Y, Fine JT, Lam J, Garrison LP. Assessing health-related quality-of-life in patients with symptomatic obstructive hypertrophic cardiomyopathy: EQ-5D-based utilities in the EXPLORER-HCM trial. J Med Econ. 2022 Jan-Dec;25(1):51-58. doi: 10.1080/13696998.2021.2011301. PMID: 34907813.

Published

18/09/2023

How to Cite

Pereira de Paiva, C., Godinho de Freitas, A. C., Germinari Souza, C. L., Tochihide Iamaguti, F., Henrique Costa Pinto , G., Peruzzi Iamaguti, J., … Mendonça Rocha, C. (2023). MAVACAMTEN IN THE TREATMENT OF HYPERTROPHIC CARDIOMYOPATHY. RECIMA21 - Revista Científica Multidisciplinar - ISSN 2675-6218, 4(9), e494044. https://doi.org/10.47820/recima21.v4i9.4044