Mavacamten in HOCM

Mavacamten for treating symptomatic obstructive hypertrophic cardiomyopathy

Mavacamten is recommended as an option for treating symptomatic obstructive hypertrophic cardiomyopathy in adults who have a New York Heart Association class of 2 or 3.It is recommended only if:

• it is an add-on to individually optimised standard care that includes beta-blockers, non-dihydropyridine calcium-channel blockers or disopyramide, unless these are contraindicated, and
• the company provides it according to the commercial arrangement

The NICE committee note:

• "..treatment for obstructive hypertrophic cardiomyopathy aims to manage its symptoms. Standard care is either beta-blockers or non-dihydropyridine calcium-channel blockers and if symptoms persist then disopyramide may be added. Some people with uncontrolled symptoms may choose to have surgery. Mavacamten is the first treatment that specifically treats the condition rather than the symptoms.."

A review states:

• mavacamten is the first cardiac myosin inhibitor approved for the treatment of adults with symptomatic obstructive hypertrophic cardiomyopathy (2)
  • provides a novel pharmacologic treatment option for patients, which targets the underlying pathophysiology of the disease, and it is well tolerated in a large majority of patients
  • ased on the available data, mavacamten is beneficial, at least in the short to medium term, in patients with obstructive hypertrophic cardiomyopathy who remain symptomatic despite single or multi-drug dose treatment with beta-blockers and calcium channel blockers and may postpone or avoid the need for SRT (septal reduction therapy)

Reference:

• NICE (September 2023). Mavacamten for treating symptomatic obstructive hypertrophic cardiomyopathy
• Braunwald E et al. Mavacamten: a first-in-class myosin inhibitor for obstructive hypertrophic cardiomyopathy, European Heart Journal, 2023;, ehad637, https://doi.org/10.1093/eurheartj/ehad637