Mavacamten treatment in symptomatic obstructive hypertrophic cardiomyopathy patients led to significant improvement in LVOT obstruction and exercise capacity compared to placebo.
Actively implementing contemporary management strategies, including ICDs, non-vitamin K antagonist oral anticoagulants, and therapies for LVOT obstruction and heart failure, can substantially reduce morbidity and mortality in hypertrophic cardiomyopathy.
Hypertrophic cardiomyopathy (HCM) is one of the most common inheritable cardiomyopathies. Contemporary management strategies, including the advent of implantable cardioverter-defibrillators and effective anticoagulation, have substantially improved the clinical course of HCM patients; however, the disease burden of HCM is still high in Korea. Sudden cardiac death (SCD), atrial fibrillation and thromboembolic risk, dynamic left ventricular outflow tract (LVOT) obstruction, and heart failure (HF) progression remain important issues in HCM. SCD in HCM can be effectively prevented with implantable cardioverter-defibrillators. However, appropriate patient selection is important for primary prevention, and the 5-year SCD risk score and the presence of major SCD risk factors should be considered. Anticoagulation should be initiated in all HCM patients with atrial fibrillation regardless of the CHA2DS2-VASc score, and non-vitamin K antagonist oral anticoagulants are the first option. Symptomatic dynamic LVOT obstruction is first treated medically with negative inotropes, and if symptoms persist, septal reduction therapy is considered. The recently approved myosin inhibitor mavacamten is promising. HF in HCM is usually related to diastolic dysfunction, while about 5% of HCM patients show reduced left ventricular ejection fraction <50%, also referred to as "end-stage" HCM. Myocardial fibrosis plays an important role in the progression to advanced HF in patients with HCM. Patients who do not respond to guideline-directed medical therapy can be considered for heart transplantation. The development of imaging techniques, such as myocardial deformation on echocardiography and late gadolinium enhancement on cardiac magnetic resonance, can provide better risk evaluation and decision-making for management strategies in HCM.
Lee et al. (Sat,) conducted a other in Hypertrophic Cardiomyopathy. mavacamten vs. placebo was evaluated on improvement in LVOT obstruction, subjective symptoms, and exercise capacity. Mavacamten treatment in symptomatic obstructive hypertrophic cardiomyopathy patients led to significant improvement in LVOT obstruction and exercise capacity compared to placebo.