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A Comparative Study of the Effects of Selective Myosin Inhibitors Mavacamten and Cibenzoline on Exercise Tolerance in Patients with Obstructive Hypertrophic Cardiomyopathy

Basic Information

Recruitment status	Recruiting
Health condition(s) or Problem(s) studied	Obstructive hypertrophic cardiomyopathy
Date of first enrollment	24/04/2026
Target sample size	45
Countries of recruitment
Study type	Interventional
Intervention(s)	In patients with obstructive hypertrophic cardiomyopathy, cardiopulmonary exercise testing will be performed before and after switching from sivenzolin to mavacamten to evaluate exercise tolerance.

Outcome(s)

Primary Outcome

Difference (change value) between peak VO2 measured during baseline cardiopulmonary exercise testing while taking sibenzoline and peak VO2 measured 40 weeks after switching to oral administration of mabacamten.

Secondary Outcome

1. Change in pressure gradient across the left ventricular outflow tract during the Valsalva maneuver before and after mabacumten administration 2. Change in NT-pro BNP before and after mabacumten administration 3. Change in KCCQ-12 before and after mabacumten administration 4. Change in NYHA class before and after Mabacamten administration 5. Change in exercise tolerance indices from cardiopulmonary exercise testing: OUES (oxygen uptake efficiency slope), anaerobic threshold AT, VE vs. VCO2 slope, Max ETCO2, peak workload Peak WR, peak heart rate Peak HR, R (gas exchange ratio), oxygen uptake/work rate (aerobic capacity), peak VO2/HR, peak circulatory power, ventilatory power 6. Changes in grip strength and skeletal muscle index before and after Mabacamten administration 7. Correlation between baseline NT-pro BNP, left ventricular outflow tract pressure gradient, Hypertrophic Cardiomyopathy -Sudden Cardiac Death Risk model score, and peak VO2change 8. Safety evaluation items: Incidence of adverse events

Key inclusion & exclusion criteria

Age minimum	>= 20age old
Age maximum	Not applicable
Gender	Both
Include criteria	1. Patients aged 20 years or older 2. Patients with symptomatic obstructive hypertrophic cardiomyopathy (left ventricular maximum wall thickness >=15 mm, or >=13 mm with a family history, and left ventricular pressure gradient >=30 mmHg at rest or during exercise) 3. Patients with a baseline left ventricular ejection fraction (LVEF) >=55% on echocardiography 4.Patients taking sibenzoline for at least 2 weeks in addition to beta-blockers or calcium channel blockers at baseline 5. Individuals for whom consent for participation in this study was obtained via signed informed consent form by the subject or legal representative 6. Patients capable of undergoing cardiopulmonary exercise testing using a seated cycle ergometer
Exclude criteria	1. Patients with a history of drug hypersensitivity to macamute and for whom continued oral administration of macamute is considered difficult. 2. Patients with a history of syncope or sustained ventricular tachycardia during exertion within 6 months prior to screening. 3. Patients with a history of cardiac arrest or appropriate implantable cardioverter-defibrillator (ICD) activation within 6 months prior to screening. 4.Patients with persistent atrial fibrillation at screening who have not received anticoagulant therapy for at least 4 weeks or who have not achieved adequate rate control (heart rate > 110 beats/min). 5. Patients who underwent septal ablation therapy within 6 months prior to screening. 6.Patients with NYHA functional class IV. 7. Patients with Child-Pugh class C hepatic impairment. 8. Patients with symptomatic chronic obstructive pulmonary disease or symptomatic restrictive pulmonary disease. 9. Breastfeeding women. 10. Pregnant women or women who may be pregnant. 11.Patients presenting cardiac hypertrophy associated with the following conditions: Hypertensive heart disease, valvular heart disease, congenital heart disease, ischemic heart disease, endocrine heart disease, anemia, cor pulmonale, patients with specific cardiomyopathies* 12. Other patients deemed inappropriate for study participation by the principal investigator (or co-investigator)