mavacamten: Add 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: Administer an appropriate dose of beta-blockers according to the patient's tolerance.
diltiazem: Administer an appropriate dose of diltiazem according to the patient's tolerance.
Study summary
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.
Eligibility
Sex
ALL
Min age
18 Years
Max age
75 Years
Healthy volunteers
No
Inclusion Criteria:
* Patients diagnosed with HCM according to the 2023 Chinese Guidelines for the Diagnosis and Treatment of Adult Hypertrophic Cardiomyopathy, meeting one of the following:
1. Left ventricular wall thickness ≥15 mm at end-diastole in any segment as assessed by echocardiography or cardiac magnetic resonance imaging (CMR);
2. Left ventricular wall thickness ≥13 mm in individuals with a confirmed pathogenic gene mutation or in genetically affected family members;
3. Exclusion of other cardiovascular, systemic, or metabolic disorders that may cause ventricular hypertrophy.
* Symptomatic non-outflow tract obstructive HCM patients (meeting criterion a and at least one of b or c):
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1. Presence of clinical symptoms such as dyspnea, chest pain, dizziness, palpitations, or syncope, with New York Heart Association (NYHA) functional class II-III;
2. Maximal pressure gradient (PGmax) \>30 mmHg in the mid-ventricle under resting or Valsalva maneuver as assessed by echocardiography;
3. PGmax \>30 mmHg in the apical region under resting or Valsalva maneuver on echocardiography.
③Ability to provide written informed consent (ICF) and any required privacy authorization prior to study enrollment.
Exclusion Criteria:
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* 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.
Primary outcome measure(s)
Percentage change in pressure gradient during the Valsalva maneuver — Week 36 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.
Trial sites (1)
Facility
City
Region
Status
Second Affiliated Hospital, Zhejiang University School of Medicine
Hangzhou
Zhejiang
More Second Affiliated Hospital, Zhejiang University, School of Medicine trials in China
This page summarises publicly available registry data for informational purposes — not medical advice. Eligibility is determined by each study team; patients should discuss participation with their clinician.
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