The association of aortic stenosis (AS) and obstructive hypertrophic cardiomyopathy raises questions about AS quantification, the management of left ventricular outflow tract obstruction, and AS treatment. Recently, mavacamten demonstrated its efficacy in reducing left ventricular outflow tract obstruction gradient. This case reports on mavacamten use in this challenging association.
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http://dx.doi.org/10.1016/j.jaccas.2024.102430 | DOI Listing |
Am J Health Syst Pharm
November 2024
UC San Diego Skaggs School of Pharmacy and Pharmaceutical Sciences, La Jolla, CA, USA.
Disclaimer: In an effort to expedite the publication of articles, AJHP is posting manuscripts online as soon as possible after acceptance. Accepted manuscripts have been peer-reviewed and copyedited, but are posted online before technical formatting and author proofing. These manuscripts are not the final version of record and will be replaced with the final article (formatted per AJHP style and proofed by the authors) at a later time.
View Article and Find Full Text PDFESC Heart Fail
October 2024
Division of Cardiovascular Sciences, School of Medical Sciences, Faculty of Biology, Medicine and Health, Manchester Academic Health Science Centre, University of Manchester, Oxford Road, Manchester, M13 9PL, UK.
J Physiol
October 2024
Department of Biomedical Sciences, University of Copenhagen, Copenhagen, Denmark.
Nemaline myopathy (NM) is a genetic muscle disease, primarily caused by mutations in the NEB gene (NEB-NM) and with muscle myosin dysfunction as a major molecular pathogenic mechanism. Recently, we have observed that the myosin biochemical super-relaxed state was significantly impaired in NEB-NM, inducing an aberrant increase in ATP consumption and remodelling of the energy proteome in diseased muscle fibres. Because the small-molecule Mavacamten is known to promote the myosin super-relaxed state and reduce the ATP demand, we tested its potency in the context of NEB-NM.
View Article and Find Full Text PDFJACC Case Rep
August 2024
Department of Cardiology, Amiens University Hospital, Amiens, France.
The association of aortic stenosis (AS) and obstructive hypertrophic cardiomyopathy raises questions about AS quantification, the management of left ventricular outflow tract obstruction, and AS treatment. Recently, mavacamten demonstrated its efficacy in reducing left ventricular outflow tract obstruction gradient. This case reports on mavacamten use in this challenging association.
View Article and Find Full Text PDFCurr Cardiol Rep
September 2024
Heart and Vascular Institute, Pennsylvania State Health Milton S. Hershey Medical Center, Hershey, PA, USA.
Purpose Of Review: There has been much debate surrounding novel medical therapies and heart transplantation listing challenges in patients with hypertrophic cardiomyopathy (HCM).
Recent Findings: Recent clinical trials led to FDA approval of mavacamten (a cardiac myosin inhibitor), offering symptom relief and potentially delaying/avoiding invasive septal reduction therapies for some patients with HCM and left ventricular outflow obstruction (LVOTO). For those with refractory symptoms and end-stage heart failure, heart transplantation remains the gold standard.
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