This study is a prospective interventional cohort study aimed at evaluating the therapeutic efficacy and clinical utility of Mavacamten-a targeted myosin inhibitor specifically developed for obstructive hypertrophic cardiomyopathy (HCM)-in patients with HCM characterized by mid-to-apical left ventricular obstruction.
Left ventricular wall thickness ≥15 mm at end-diastole in any segment as assessed by echocardiography or cardiac magnetic resonance imaging (CMR);
Left ventricular wall thickness ≥13 mm in individuals with a confirmed pathogenic gene mutation or in genetically affected family members;
Exclusion of other cardiovascular, systemic, or metabolic disorders that may cause ventricular hypertrophy.
Presence of clinical symptoms such as dyspnea, chest pain, dizziness, palpitations, or syncope, with New York Heart Association (NYHA) functional class II-III;
Obstructive hypertrophic cardiomyopathy (HCM), defined as a maximal left ventricular outflow tract pressure gradient (LVOT-PGmax) ≥30 mmHg at rest and during the Valsalva maneuver on echocardiography;
Maximal right ventricular outflow tract pressure gradient (RVOT-PGmax) ≥16 mmHg at rest; ③ Left ventricular ejection fraction (LVEF) <50% on echocardiography;
Uncontrolled primary hypertension;
Moderate or severe aortic valve stenosis and/or primary mitral valve disease with severe mitral regurgitation; ⑥ Known infiltrative or storage disorders mimicking the HCM phenotype (e.g., Fabry disease, cardiac amyloidosis); ⑦ Presence of severe infections, hepatic dysfunction, renal impairment, or other serious conditions significantly affecting life expectancy.
Parallel
mavacamten
DrugAdd Mavacamten to guideline-directed standard medical therapy for patients with hypertrophic cardiomyopathy (HCM) and mid-to-apical left ventricular obstruction.
Beta Blocker (BB) - metoprolol, bisoprolol, carvedilol
DrugAdminister an appropriate dose of beta-blockers according to the patient's tolerance.
diltiazem
DrugAdminister an appropriate dose of diltiazem according to the patient's tolerance.
Percentage change in pressure gradient during the Valsalva maneuver
The percentage reduction in the pressure gradient at the site of left ventricular obstruction during the Valsalva maneuver at week 36, compared to baseline, as measured by echocardiography.
Anterior papillary muscle to the interventricular septum distance
Percentage Increase in the distance from the anterior papillary muscle to the interventricular septum
Left atrial global longitudinal strain
Left Atrial Global Longitudinal Strain (LA GLS) is an echocardiographic parameter derived from speckle-tracking imaging that measures the longitudinal deformation of the left atrial myocardium throughout the cardiac cycle.
Left ventricular global longitudinal strain
Left Ventricular Global Longitudinal Strain (LVGLS) is a sensitive echocardiographic parameter derived from speckle-tracking imaging that quantifies the myocardial deformation in the longitudinal direction. It reflects the shortening of myocardial fibers along the long axis of the left ventricle during systole.
Percentage change in resting pressure gradient
The percentage reduction in the pressure gradient at the site of left ventricular obstruction at rest measured by echocardiography at week 36 compared to baseline.
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