Mavacamten Enables Exercise in Hypertrophic Obstructive Cardiomyopathy

Summary

Patients with hypertrophic obstructive cardiomyopathy and New York Heart Association Class I-II under stable treatment with mavacamten (at least 12 weeks without change of dosage) and a peak left ventricular outflow tract obstruction \<50mmHg undergo either 6 weeks of structured moderate intensity endurance and resistance training (supervised, 3x/week, intervention, IT) or usual care (UC). Patients within 1 hour of travel to the training venue will be referred to IT, while those with more than 1 hour will join UC. At baseline (visit 1, V1) and after 6 weeks of exercise intervention (visit 2, V2) all patients undergo a medical exam, resting and stress echocardiography and receive a questionnaire on the quality of life (Kansas City Cardiomyopathy Questionnaire). Cardiac biomarkers are assessed. 3 hours after stress echocardiography cardiopulmonary exercise testing is performed to measure peak oxygen consumption (VO2peak). The primary outcome is safety. Secondary outcomes include the change of VO2peak, changes in cardiac biomarkers, resting and stress echocardiographic variables, quality of life and variables of cardiopulmonary exercise testing from V1 to V2.

Eligibility

Inclusion Criteria:

* Age ≥18 years of age
* Diagnosis of hypertrophic obstructive cardiomyopathy
* ≥12 weeks of unchanged dosage of mavacamten
* Peak left ventricular outflow tract gradient ≤ 50 mmHg at rest and during stress echocardiography
* Left ventricular ejection fraction ≥50% at study inclusion
* New York Heart Association classes I-II

Exclusion Criteria:

* Syncope or sustained ventricular tachycardia within 6 months prior to study inclusion
* Corrected QT-interval (Fridericia-formula) ≥ 500 ms
* Paroxysmal or intermittent atrial fibrillation (AF) on screening electrocardiogram
* Persistent or permanent AF without anticoagulation for ≥4 weeks
* Previous transcoronary ablation of septal hypertrophy or surgical myectomy
* Ventricular tachycardia, significant ST-elevation or depression upon baseline cardiopulmonary exercise testing
* ≥ grade II valve insufficiencies or stenoses during resting echocardiography
* Prior implantable cardioverter defibrillator-implantation
* Sudden Cardiac Death Risk Score ≥4%

Conditions2

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