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One- and two-year structural changes of mavacamten therapy in hypertrophic obstructive cardiomyopathy: a case report
Katharina Seuthe1, Roman Pfister1, Carl-Hubertus Schönherr2
1Department III of Internal Medicine, Heart Center, Faculty of Medicine and University Hospital Cologne, University of Cologne, Kerpener Straße 62, Cologne 50937, Germany.
Insights
Mavacamten therapy for hypertrophic obstructive cardiomyopathy (HOCM) shows sustained cardiac reverse remodeling up to two years. This myosin inhibitor improves cardiac structure and function, offering long-term benefits beyond initial symptom relief.
Area of Science:
- Cardiology
- Pharmacology
- Medical Imaging
Background:
- Hypertrophic obstructive cardiomyopathy (HOCM) involves left ventricular outflow tract (LVOT) obstruction and reduced quality of life.
- Mavacamten, a novel myosin inhibitor, is a promising therapeutic option for HOCM.
- Existing data on mavacamten's effects, particularly from cardiovascular magnetic resonance (CMR), are limited to short-term follow-up.
Background:
Hypertrophic obstructive cardiomyopathy (HOCM) is characterized by dynamic left ventricular outflow tract (LVOT) obstruction and impaired quality of life. Mavacamten, a first-in-class myosin inhibitor, offers a novel therapeutic approach for HOCM, which improves clinical symptoms and exercise capacity while leading to reduction of LVOT gradient and favourable cardiac remodelling in echocardiography and cardiovascular magnetic resonance (CMR). However, data on CMR-derived treatment effects remain limited to short follow-up studies.
Case Summary:
A 39-year-old male with symptomatic HOCM refractory to bisoprolol and disopyramide was initiated on mavacamten therapy. Serial CMR after one and two years demonstrated progressive reverse remodelling over the follow-up period, including ongoing reduction of LV ejection fraction and LV mass index. While late gadolinium enhancement mass remained unchanged, there were divergent changes to measures of interstitial fibrosis: While native T1 relaxation times steadily decreased, extracellular volume (ECV) fraction initially increased but normalized at two-year follow-up.
Discussion:
This case indicates that mavacamten leads to longer-term cardiac remodelling up to two years after initiation of treatment, beyond early haemodynamic improvement and reduction of LVOT obstruction. These observations nurture additional studies to investigate longer-term effects on myocardial structure, function, and interstitial fibrosis.
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