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Mavacamten as an Effective Treatment for HCM With Latent Exercise-Induced Obstruction: A Case Report
Nikitha Murthy1, Brian Diep1, Jonathan Evans1
1Division of Cardiology, Loma Linda University Health, Loma Linda, California, USA, lluh.org.
Insights
Mavacamten effectively treats symptomatic hypertrophic cardiomyopathy (HCM) with exercise-induced obstruction. This myosin inhibitor improved patient symptoms, even without resting obstruction, highlighting its potential in a broader HCM population.
Area of Science:
- Cardiology
- Pharmacology
Background:
- Hypertrophic cardiomyopathy (HCM) is a myocardial disorder causing left ventricular hypertrophy and symptoms, especially during exercise.
- Obstructive HCM can be treated with mavacamten, a myosin inhibitor that reduces obstruction and symptoms.
Purpose of the Study:
- To evaluate the efficacy of mavacamten in a patient with symptomatic HCM and exercise-induced left ventricular outflow tract obstruction (LVOTO).
Main Methods:
- Case presentation of a symptomatic HCM patient.
- Treadmill stress echocardiography to assess for exercise-induced LVOTO.
- Treatment with mavacamten.
Main Results:
- The patient had no resting LVOTO but demonstrated obstruction during exercise.
- Symptomatic improvement was observed after mavacamten treatment.
Conclusions:
- Symptomatic HCM patients without resting gradients may benefit from exercise stress testing to identify obstructive HCM.
- Mavacamten may be a safe and effective treatment for patients with exercise-induced LVOTO.
Background:
Hypertrophic cardiomyopathy (HCM) is a myocardial disorder characterized by hypertrophy of the left ventricle that can lead to significant symptoms, particularly with exercise. For patients with HCM who demonstrate obstructive physiology, the first-in-class targeted cardiac specific myosin inhibitor, mavacamten, has been found to reduce the degree of obstruction as well as symptoms.
Case Summary:
We present a patient with symptomatic HCM who had no left ventricular outflow tract obstruction (LVOTO) at rest but demonstrated obstruction on treadmill stress echocardiography. She had symptomatic improvement after treatment with mavacamten.
Discussion:
Patients with symptomatic HCM who do not demonstrate elevated resting gradients may benefit from evaluation of exercise-induced gradients to diagnose obstructive HCM and guide initiation of targeted therapy with mavacamten.
Take Home Message:
Mavacamten may be safe and effective in patients who demonstrate LVOTO only upon provocation with exercise.
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