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Case Reports
. 2025 May 8;9(5):ytaf229.
doi: 10.1093/ehjcr/ytaf229. eCollection 2025 May.

Efficacy of mavacamten in patients with hypertrophic cardiomyopathy and mid-ventricular obstruction: case series

Valeria Rella  1 , Denisa Muraru  2   3 , Lia Crotti  1   3
Affiliations
Case Reports

Efficacy of mavacamten in patients with hypertrophic cardiomyopathy and mid-ventricular obstruction: case series

Valeria Rella et al. Eur Heart J Case Rep. .

Abstract

Background: Mavacamten is a cardiac-specific myosin inhibitor approved for treatment of adults with hypertrophic cardiomyopathy (HCM) symptomatic for left ventricular outflow tract (LVOT) obstruction. Since obstruction is favoured by a hyper-contractile state, it would be logical to suppose that mavacamten may also be effective in patients with mid-ventricular obstruction (MVO). We present our experience with two HCM patients having MVO effectively treated with mavacamten.

Case summary: The first case is a 55-year-old woman presenting with dyspnoea and exertional fatigue, with obstructive HCM (HOCM) and mid-ventricular peak gradient of 77 mmHg associated with LVOT obstruction. The treatment with mavacamten 5 mg daily determined relief of symptoms. At 16-week follow-up, there was a significant reduction of peak gradient (11 mmHg in mid-ventricular tract) and a significant decrease in NT-proBNP levels from 1287 to 178 ng/L. The second case is a 55-year-old woman with predominant mid-ventricular HOCM (peak gradient 52 mmHg) and past history of septal myectomy, with a residual significant gradient measured at LVOT level. The patient was started on mavacamten 5 mg daily, subsequently up-titrated to 10 mg. At 16-week follow-up, there was a significant reduction of peak gradient to 10 mmHg and a significant decrease in NT-proBNP levels from 3910 to 718 ng/L.

Discussion: These two cases highlight the efficacy of mavacamten in the reduction of MVO, suggesting that it may be a valid therapeutic option also in patients with isolated MVO, frequently more difficult to be adequately treated.

Keywords: Case report; Hypertrophic cardiomyopathy; Mavacamten; Mid-ventricular obstruction.

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Conflict of interest statement

Conflict of interest. L.C. was part of the advisory board of BMS for mavacamten.

Figures

Figure 1
Figure 1
Echocardiographic characteristics at baseline and at 16 weeks follow-up in Patient 1. On the left side, baseline echocardiographic data are shown (A) while on the right side, data after 16 weeks of treatment with mavacamten are indicated (B). Note the differences in scale. LVEF, left ventricular ejection fraction; LA, left atrium.
Figure 2
Figure 2
Echocardiographic characteristics at baseline and at 16 weeks follow-up in Patient 2. On the left side baseline echocardiographic data are shown (A) while on the right side, data after 16 weeks of treatment with mavacamten are indicated (B). Note the differences in scale. LVEF, left ventricular ejection fraction; LA, left atrium.
None
See this image and copyright information in PMC

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