ABSTRACT

Introduction: Hypertrophic cardiomyopathy (HCM) is a common known monogenetic cardiovascular disorder which leads to dyspnea and exercise tolerance. Recent guidelines have different therapies. Hence, mavacamten is a small molecule modulator of beta cardiac myosin reduces hypercontractability which is the central mechanism of HCM. Hence, it is evaluated in phase 2 and 3 clinical trials for obstructive and non obstructive symptomatic HCM. Purpose of Review: The pharmacological treatment for hypertrophic cardiomyopathy are limited and includes therapies such as beta blockers, non-dihydropyridine calcium channel blockers and disopyramide. They offer variable degree of symptomatic relief, suboptimal and are limited by side effects which addresses to molecular abnormalities of the disease. Recent Finding: Mavacamten is novel, allosteric inhibitor of cardiac myosin ATPase, whichreduces actin-myosin cross bridge formation thus may reduce contractability and improves myocardial energy consumption. The phase 2 and phase 3 demonstrates the efficacy and safety in reducing left ventricular outflow tract obstruction and exercise capacity in oHCM patients. Summary: Mavacamten represent the first agent for HCM which is registered for clinical use, representing a change in paradigm in the pharmacological treatment of HCM. It is effective in obstructive HCM and non obstructive HCM. Keywords: Hypertrophic cardiomyopathy, Mavacamten, Myosin, Left ventricular outflowobstruction, Therapy
Publication date: 01/10/2024

https://ijbpas.com/pdf/2024/October/MS_IJBPAS_2024_8380.pdf

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https://doi.org/10.31032/IJBPAS/2024/13.10.8380