The objective of this observational study is to evaluate the association between the presence of mitral valvular and/or subvalvular anatomical abnormalities prior to initiation of mavacamten and the hemodynamic response at 6 months in patients with obstructive HCM treated with mavacamten. The secondary objective is to determine the impact of different types of anatomical abnormalities of the mitral valve and subvalvular structures on the hemodynamic and clinical response to mavacamten.
Age ≥18 years
Patients who are informed and consent to participate
CMH with significant obstruction (left intraventricular gradient at rest or during Valsalva maneuver ≥ 50 mmHg) and symptomatic (NYHA class 2 or 3)
Patients who have started treatment with Mavacamten
Patients who refuse to participate in this register
Patient with a diagnosis of HCM with significant obstruction (left intraventricular gradient at rest or during Valsalva maneuver ≥ 50 mmHg) and symptomatic (NYHA class 2 or 3) and started treatment with Mavacamten
Cohort
Retrospective
Not listed
Change in the highest left ventricular outflow tract (LVOT) Doppler maximal gradient (rest or Valsalva) from baseline to 6 months after initiation of mavacamten
Comparison of the change in LVOT gradient according to the presence or absence of mitral valvular and/or subvalvular anatomical abnormalities at baseline
Change in NYHA functional class
The association between the type of baseline mitral valvular and/or subvalvular anatomical abnormalities and clinical response will be assessed
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