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.
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
TREATMENT
Masking
NONE
Enrollment
132
Add Mavacamten to guideline-directed standard medical therapy for patients with hypertrophic cardiomyopathy (HCM) and mid-to-apical left ventricular obstruction.
Administer an appropriate dose of beta-blockers according to the patient's tolerance.
Administer an appropriate dose of diltiazem according to the patient's tolerance.
Second Affiliated Hospital, Zhejiang University School of Medicine
Hangzhou, Zhejiang, China
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.
Time frame: Week 36
Anterior papillary muscle to the interventricular septum distance
Percentage Increase in the distance from the anterior papillary muscle to the interventricular septum
Time frame: Week 36
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.
Time frame: Week 36
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.
Time frame: Week 36
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.
Time frame: Week 36
Absolute change in pressure gradient during the Valsalva maneuver
The absolute decrease in number of the pressure gradient at the site of left ventricular obstruction during the Valsalva maneuver measured by echocardiography at week 36 compared to baseline.
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Time frame: Week 36
The proportion of patients with a pressure gradient <30 mmHg during the Valsalva maneuver
The proportion of patients with a pressure gradient \<30 mmHg at the site of obstruction during the Valsalva maneuver at week 36.
Time frame: Week 36
BNP
Number decrease in BNP levels from baseline to week 36.
Time frame: Week 36
Troponin T
Number decrease in Troponin T levels from baseline to week 36.
Time frame: Week 36
New-onset atrial fibrillation
Rate of new-onset atrial fibrillation in each group during the study period
Time frame: From baseline to week 36
LVEF<50%
The proportion of patients in each group with LVEF \<50% accompanied by signs and symptoms of heart failure worsening and/or an increase in BNP of ≥30% compared to the previous measurement during the study period.
Time frame: From baseline to week 36
LVEF<40%
Rate of LVEF \<40% in each group during the study period.
Time frame: From baseline to week 36