Mavacamten treatment in obstructive HCM patients resulted in significant LVOT gradient reductions of -13.1 mmHg at rest and -29.1 mmHg with provocation after one month.
Does mavacamten improve echocardiographic parameters and NYHA class in symptomatic patients with obstructive hypertrophic cardiomyopathy?
In a real-world setting, one month of mavacamten therapy in symptomatic HOCM patients led to significant early improvements in NYHA class and reductions in LVOT gradients and mitral regurgitation.
Absolute Event Rate: 0% vs 0%
Abstract Background Hypertrophic cardiomyopathy (HCM) is a genetic cardiac disease primarily caused by sarcomere gene mutations. It is characterized by left ventricular hypertrophy and, in many cases, left ventricular outflow tract (LVOT) obstruction. Mavacamten, a cardiac myosin inhibitor, reduces myocardial contractility and has shown efficacy in relieving obstruction-related symptoms. Objective To evaluate clinical and echocardiographic changes after one month of mavacamten therapy in symptomatic obstructive HCM (HOCM) patients in routine clinical practice. Methods This prospective study included 11 symptomatic patients treated with mavacamten in a large referral center. Clinical data (NYHA class) and echocardiographic parameters were assessed at baseline and after one month. Parameters analyzed included LVOT gradient (rest and provocation), global left ventricular (LV) and left atrial (LA) longitudinal strain (GLS), regional LA strain (reservoir, conduit, contraction), indexed LA volume (LAVI), E/E’, pulmonary artery systolic pressure (PASP), maximal wall thickness (MWT), left ventricular ejection fraction (LVEF), right ventricular free wall strain, myocardial work indices; constructive work (CWG), Global Longitudinal Strain (GLS), global work index (GWI), and hemodynamic forces (apex-base and lateral-septal). Paired t-tests were used for comparison. Results Mean patient age was 63 years (range 50–81), with 45% female. 5 patients were genetically tested and 2 (40%) were positive (MYH7). Baseline NYHA class was 2.82 and baseline LVOTO was 41.55. After one month of mavacamten use, NYHA class significantly improved (final FC 2.00; p = 0.046) and LVOT gradient decreased significantly both at rest (-13.1 mmHg, p = 0.008) and with provocation (-29.1 mmHg, p = 0.018). Mitral regurgitation severity improved (-2 grades, p = 0.005). A modest but significant reduction in LVEF was noted (p = 0.018), while both LV GLS and global constructive work (GCW) showed a tendency of improvement (p = 0.052 and 0.075 respectively). Trends toward diastolic improvement were observed in E/e’ ratio (p = 0.093), LAVI (p = 0.078) and left atrial conduct strain (p = 0.068). MWT remained unchanged. No adverse events or treatment discontinuations were reported. (Table 1). Conclusions After one month of mavacamten therapy, patients showed early clinical and echocardiographic improvements, including marked reductions in LVOT gradients and mitral regurgitation. While LVEF decreased, LV strain and constructive work improved, suggesting a more efficient myocardial contraction, with a trend for improved diastolic function. These findings support the early therapeutic benefit of mavacamten in real-world HOCM patients.
Videla et al. (Thu,) reported a other. Mavacamten treatment in obstructive HCM patients resulted in significant LVOT gradient reductions of -13.1 mmHg at rest and -29.1 mmHg with provocation after one month.
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