Mavacamten, a novel revolutionizing therapy in hypertrophic obstructive cardiomyopathy: A literature review

Autores da FMUP

• Elisabete Lousada Martins Oliveira Bernardes

  Autor

Participantes de fora da FMUP

• Aguiar, T

Unidades de investigação

• Laboratório Associado RISE- Rede de Investigação em Saúde

  

  Investigador

  Fernando Carlos De Landér Schmitt

• Medicina

• RISE-Health

  

  Investigador

  Fernando Carlos De Landér Schmitt

Abstract

Hypertrophic cardiomyopathy (HCM) is one of the most common inherited cardiac diseases, defined as a left ventricular wall thickness of >15 mm, in the absence of other causes of abnormal ventricular loading. A major hallmark of this disease is the presence of left ven-tricular outflow tract obstruction, which develops in up to three quarters of patients, referred to as obstructive hypertrophic cardiomyopathy. Current treatment is offered to symptomatic patients, based on the presence of documented left ventricular obstruction, aimed at reducing symptoms and disease progression. This is achieved through pharmacological empirical ther-apy, surgery, alcohol ablation and/or pacing. Mavacamten is a first-in-class allosteric inhibitor of cardiac myosin that promises to provide clinicians with targeted therapy for these patients. The aim of this review is to provide a general overview of the modern approach to the diagnosis and management of HCM, as well as to integrate all the current knowledge on mavacamten, in anticipation of a future change in the treatment algorithm of patients with HCM. (c) 2022 Sociedade Portuguesa de Cardiologia. Published by Elsevier Espan similar to a, S.L.U. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Keywords

• MYK-461; Hypertrophic cardiomyopathy; Left ventricular outflow obstruction