hypertrophic cardiomyopathy
Conditions
Interventions
Patients diagnosed with obstructive hypertrophic cardiomyopathy and treated with myocardial myosin inhibitors as recommended by guidelines:NA
Sponsors
West China Hospital of Sichuan University
Eligibility
Sex/Gender
All
Age
18 Years to No maximum
Inclusion criteria
Inclusion criteria: 1) Age >=18 years old; 2) Hypertrophic cardiomyopathy was diagnosed; 3) Adequate acoustic window for accurate echocardiography; 4) LVOT peak pressure difference >=50mmHg at rest or excitation; 5) Echocardiography showed LVEF >=55% at rest; 6) New York Heart Association (NYHA) Grade II-III cardiac function scale 7) Left ventricular outflow tract obstruction leading to persistent symptoms despite the use of beta-blockers or non-dihydropyridine calcium channel blockers 8) Receive beta-blockers or non-dihydropyridine calcium channel blockers at steady doses for more than 6 weeks and are expected to remain on the same drug regimen during the study period. 9) Be able to follow and understand the research process, and be willing to sign the informed consent for this project
Exclusion criteria
Exclusion criteria: 1) myocardial hypertrophy caused by other causes; 2) ventricular septal volume reduction therapy (interventricular myocardial resection or percutaneous alcohol interventricular septal ablation, etc.) was successfully received in the past or planned during the study period; 3) Past use of myocardial myosin inhibitors; 4) Known allergy to any component of the mavacamten formulation; 5) Receiving CYP2C19 inhibitors or moderate-to-strong CYP3A4 inhibitors; 6) The female subject is pregnant or lactating; 7) Subjects plan pregnancy during the study period; 8) Failure to complete a cardiac MRI scan (there are contraindications to cardiac MRI, such as claustrophobia, etc.) or poor quality or missing cardiac MRI images;
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Diameter and volume of left and right ventricles and atria, ejection fraction of left and right ventricles, myocardial fibrosis evaluation based on LEG, ventricular extracellular volume (ECV) evaluation using T1 mapping imaging and post-processing, and myocardial mechanical and functional changes were evaluated using feature-tracking technology. HDF and PV loop were used to evaluate hemodynamic changes, and mOEF was used to evaluate myocardial oxygen consumption;Peak V 'O2, V 'e /V 'CO2, RER, and corresponding predicted percentages; | — |
Secondary
| Measure | Time frame |
|---|---|
| Blood routine, blood biochemistry, troponin, NT-pro-BNP;Left and right ventricle and atrium diameter, ventricular wall thickness, left ventricular ejection fraction, tricuspid annular plane systolic displacement (TAPSE), TDI-S, valve morphology and opening and closing, blood flow regurgination and acceleration in each valve area, subvalvular structure and function, abnormal blood flow acceleration in the heart cavity, E/A, E/Ea.; | — |
Countries
China
Contacts
Public ContactChen Yucheng
West China Hospital of Sichuan University
Outcome results
None listed