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The Central Role of Echocardiography in Mavacamten Therapy for Obstructive Hypertrophic Cardiomyopathy: A
Ali Nizam1, Ibrahim Antoun1,2, Liz Hodson1
1Department of Cardiology, University Hospitals of Leicester NHS Trust, Glenfield Hospital, Leicester, UK, nhs.uk.
Insights
Mavacamten therapy for obstructive hypertrophic cardiomyopathy (HCM) relies heavily on echocardiography for patient selection and monitoring. Standardized echocardiographic assessments are crucial for effective mavacamten treatment and accurate physiological response evaluation.
Area of Science:
- Cardiology
- Medical Imaging
- Pharmacology
Background:
- Obstructive hypertrophic cardiomyopathy (HCM) is a significant cardiovascular condition.
- Mavacamten, a cardiac myosin inhibitor, offers a therapeutic option for symptomatic obstructive HCM.
- Echocardiography plays a pivotal role in the management of patients treated with mavacamten.
Purpose of the Study:
- To provide a practical echocardiographic approach for mavacamten therapy.
- To emphasize key echocardiographic parameters for patient selection, monitoring, and dose titration.
- To discuss current regulatory requirements and unresolved issues in echocardiographic assessment for mavacamten.
Main Methods:
- Narrative review of echocardiographic practices in mavacamten therapy.
- Focus on reproducible left ventricular outflow tract (LVOT) gradient assessment.
- Inclusion of Valsalva manoeuvres, LVEF monitoring, and assessment of mitral valve dynamics.
Main Results:
- Echocardiography is essential for identifying suitable patients and guiding mavacamten treatment.
- Regulatory guidelines mandate specific echocardiographic monitoring during titration and maintenance.
- Standardized echocardiographic acquisition and reporting are vital for reliable treatment decisions.
Conclusions:
- A standardized echocardiographic approach is necessary for effective mavacamten therapy in obstructive HCM.
- Consistent monitoring ensures treatment decisions reflect true physiological response.
- Further research is needed for exercise echocardiography and other emerging parameters in mavacamten management.
Abstract:
Mavacamten is a cardiac myosin inhibitor used in adults with symptomatic obstructive hypertrophic cardiomyopathy (HCM). Echocardiography is central to selecting suitable patients, confirming left ventricular outflow tract (LVOT) obstruction, excluding inappropriate use when left ventricular ejection fraction (LVEF) is < 55% and guiding dose titration during treatment. This narrative review summarises the practical echocardiographic approach to mavacamten therapy, with emphasis on reproducible LVOT gradient assessment, goal-directed Valsalva manoeuvres, LVEF monitoring, mitral valve and systolic anterior motion assessment, mitral regurgitation, diastolic function and left atrial size. Current regulatory monitoring requires echocardiographic assessment during early titration and permits 6-monthly maintenance surveillance only in stable patients meeting defined criteria, including LVEF ≥ 55% and Valsalva LVOT gradient < 30 mmHg, or a decision not to uptitrate. The review also discusses unresolved issues, including exercise echocardiography, global longitudinal strain, atrial fibrillation, reverse remodelling, implementation variability and the broader emergence of cardiac myosin inhibitors such as aficamten. Standardised acquisition and reporting are essential to ensure that treatment decisions reflect true physiological response rather than technical variability.
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