{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T16:45:08Z","timestamp":1787589908910,"version":"build-2736575974"},"reference-count":17,"publisher":"BMJ","issue":"1","license":[{"start":{"date-parts":[[2025,3,28]],"date-time":"2025-03-28T00:00:00Z","timestamp":1743120000000},"content-version":"unspecified","delay-in-days":86,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Open Heart"],"accepted":{"date-parts":[[2025,2,24]]},"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:sec>\n                  <jats:title>Aims<\/jats:title>\n                  <jats:p>Mavacamten, the first approved myosin inhibitor for symptomatic obstructive hypertrophic cardiomyopathy (oHCM), addresses hypercontractility and left ventricular outflow tract (LVOT) obstruction. This study evaluates real-world experience with mavacamten, focusing on maintenance dose determination to optimise individual therapy and enhance patient safety.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>36 patients with symptomatic oHCM who completed the initiating phase of mavacamten therapy were analysed. <jats:italic>CYP2C19<\/jats:italic> genetic testing determined metabolic status prior to treatment. Echocardiographic measurements (eg, LVOT gradient, left atrial volume index, left ventricular ejection fraction (LVEF) and E\/E\u2019) and biomarkers (high-sensitivity troponin I, N-terminal pro B-type natriuretic peptide (NT-proBNP)) were assessed at baseline and after 3\u2009months. Clinical status was evaluated using New York Heart Association (NYHA) class and Kansas City Cardiomyopathy Questionnaire (KCCQ) score.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>The mean age of patients was 60.6\u00b112.1, and all had normal <jats:italic>CYP2C19<\/jats:italic> metabolic status. LVEF was 68% (IQR 8) at baseline and decreased mildly to 60.5% (IQR 7.25; p=0.0004) without cases dropping below 50%. Resting and provoked LVOT gradients decreased from 65 mm Hg (IQR 43.75) and 105 mm Hg (IQR 36.25) to 12\u2009mm Hg (IQR 15.5; p&lt;0.001) and 52.5 mm Hg (IQR 46.5; p&lt;0.001), respectively. NT-proBNP and high-sensitivity troponin I decreased significantly from 1040 ng\/mL (IQR 1255) to 285\u2009ng\/mL (IQR 483; p=0.0005) and from 11 ng\/mL (IQR 15.5) to 10\u2009ng\/mL (IQR 5; p&lt;0.0001). Diastolic function improved slightly; and clinically, patients improved significantly, with improvement in NYHA class and increase in KCCQ score. Mean time to reach maintenance dose was 14 weeks, with the necessity of dose adjustments in more than 50% of cases.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusion<\/jats:title>\n                  <jats:p>Mavacamten therapy is safe and effective in the initiating phase. Determination of starting and maximum dose is based on <jats:italic>CYP2C19<\/jats:italic> metabolic status, while individualised dose adjustments are guided by echocardiographic response to optimise patient safety.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1136\/openhrt-2025-003192","type":"journal-article","created":{"date-parts":[[2025,3,28]],"date-time":"2025-03-28T07:31:38Z","timestamp":1743147098000},"page":"e003192","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":9,"title":["Mavacamten maintenance dose determination: insights into individualised therapy for hypertrophic cardiomyopathy"],"prefix":"10.1136","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7445-5986","authenticated-orcid":false,"given":"Smita","family":"Scholtz","sequence":"first","affiliation":[{"name":"Clinic for General and Interventional Cardiology\/Angiology, Herz- und Diabeteszentrum NRW, Ruhr-Universit\u00e4t Bochum, Bad Oeynhausen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C\u00e9dric","family":"Copp\u00e9e","sequence":"additional","affiliation":[{"name":"Clinic for General and Interventional Cardiology\/Angiology, Herz- und Diabeteszentrum NRW, Ruhr-Universit\u00e4t Bochum, Bad Oeynhausen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kawa","family":"Mohemed","sequence":"additional","affiliation":[{"name":"Clinic for General and Interventional Cardiology\/Angiology, Herz- und Diabeteszentrum NRW, Ruhr-Universit\u00e4t Bochum, Bad Oeynhausen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Max","family":"Potratz","sequence":"additional","affiliation":[{"name":"Clinic for General and Interventional Cardiology\/Angiology, Herz- und Diabeteszentrum NRW, Ruhr-Universit\u00e4t Bochum, Bad Oeynhausen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vasco","family":"Sequeira","sequence":"additional","affiliation":[{"name":"Comprehensive Heart Failure Center (CHFC), University Clinic W\u00fcrzburg, W\u00fcrzburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5385-6839","authenticated-orcid":false,"given":"Volker","family":"Rudolph","sequence":"additional","affiliation":[{"name":"Clinic for General and Interventional Cardiology\/Angiology, Herz- und Diabeteszentrum NRW, Ruhr-Universit\u00e4t Bochum, Bad Oeynhausen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Werner","family":"Scholtz","sequence":"additional","affiliation":[{"name":"Clinic for General and Interventional Cardiology\/Angiology, Herz- und Diabeteszentrum NRW, Ruhr-Universit\u00e4t Bochum, Bad Oeynhausen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan-Christian","family":"Reil","sequence":"additional","affiliation":[{"name":"Clinic for General and Interventional Cardiology\/Angiology, Herz- und Diabeteszentrum NRW, Ruhr-Universit\u00e4t Bochum, Bad Oeynhausen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2025,3,28]]},"reference":[{"key":"2025081612014876000_12.1.e003192.1","doi-asserted-by":"crossref","first-page":"e1239","DOI":"10.1161\/CIR.0000000000001250","article-title":"2024 AHA\/ACC\/AMSSM\/HRS\/PACES\/SCMR Guideline for the Management of Hypertrophic Cardiomyopathy: A Report of the American Heart Association\/American College of Cardiology Joint Committee on Clinical Practice Guidelines","volume":"149","author":"Ommen","year":"2024","journal-title":"Circulation"},{"key":"2025081612014876000_12.1.e003192.2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehad194"},{"key":"2025081612014876000_12.1.e003192.3","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa021332"},{"key":"2025081612014876000_12.1.e003192.4","doi-asserted-by":"publisher","DOI":"10.1126\/science.aad3456"},{"key":"2025081612014876000_12.1.e003192.5","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(20)31792-X"},{"key":"2025081612014876000_12.1.e003192.6","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2022.04.048"},{"key":"2025081612014876000_12.1.e003192.7","unstructured":"CAMZYOS\u00ae Hartkapseln . 2024. Available: https:\/\/fi.b-ms.de\/camzyos [Accessed 30 Sep 2024]."},{"key":"2025081612014876000_12.1.e003192.8","unstructured":"Available: https:\/\/www.ema.europa.eu\/en\/documents\/product-information\/camzyos-epar-product-information_en.pdf [Accessed 11 Nov 2024]."},{"key":"2025081612014876000_12.1.e003192.9","doi-asserted-by":"crossref","first-page":"1298","DOI":"10.1111\/cts.12830","article-title":"CYP2C19 Allele Frequencies in Over 2.2 Million Direct\u2010to\u2010Consumer Genetics Research Participants and the Potential Implication for Prescriptions in a Large Health System","volume":"13","author":"Ionova","year":"2020","journal-title":"Clinical Translational Sci"},{"key":"2025081612014876000_12.1.e003192.10","doi-asserted-by":"crossref","unstructured":"Ramonfaur D , Gasperetti A , Blake VE , et al . Eighteen\u2010Month Real\u2010World Experience Using Mavacamten for Treatment of Obstructive Hypertrophic Cardiomyopathy in a Racially Diverse Population. JAHA 2024;13. doi:10.1161\/JAHA.123.034069","DOI":"10.1161\/JAHA.123.034069"},{"key":"2025081612014876000_12.1.e003192.11","doi-asserted-by":"crossref","first-page":"672","DOI":"10.1002\/ehf2.14882","article-title":"Mavacamten in real-life practice: Initial experience at a hypertrophic cardiomyopathy centre","volume":"12","author":"Abood","year":"2025","journal-title":"ESC Heart Fail"},{"key":"2025081612014876000_12.1.e003192.12","doi-asserted-by":"crossref","DOI":"10.3389\/fcvm.2024.1429230","article-title":"One-year real-world experience with mavacamten and its physiologic effects on obstructive hypertrophic cardiomyopathy","volume":"11","author":"Kim","year":"2024","journal-title":"Front Cardiovasc Med"},{"key":"2025081612014876000_12.1.e003192.13","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1016\/j.pcad.2024.02.001","article-title":"Real-world experience with mavacamten in obstructive hypertrophic cardiomyopathy: Observations from a tertiary care center","volume":"86","author":"Desai","year":"2024","journal-title":"Prog Cardiovasc Dis"},{"key":"2025081612014876000_12.1.e003192.14","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1016\/j.jchf.2023.09.028","article-title":"Mavacamten Treatment for Symptomatic Obstructive Hypertrophic Cardiomyopathy: Interim Results From the MAVA-LTE Study, EXPLORER-LTE Cohort","volume":"12","author":"Rader","year":"2024","journal-title":"JACC Heart Fail"},{"key":"2025081612014876000_12.1.e003192.15","doi-asserted-by":"crossref","first-page":"14142","DOI":"10.1021\/acs.jmedchem.1c01290","article-title":"Discovery of Aficamten (CK-274), a Next-Generation Cardiac Myosin Inhibitor for the Treatment of Hypertrophic Cardiomyopathy","volume":"64","author":"Chuang","year":"2021","journal-title":"J Med Chem"},{"key":"2025081612014876000_12.1.e003192.16","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1016\/j.jacc.2022.10.020","article-title":"Phase 2 Study of Aficamten in Patients With Obstructive Hypertrophic Cardiomyopathy","volume":"81","author":"Maron","year":"2023","journal-title":"J Am Coll Cardiol"},{"key":"2025081612014876000_12.1.e003192.17","doi-asserted-by":"crossref","DOI":"10.1161\/JAHA.124.035993","article-title":"Dosing and Safety Profile of Aficamten in Symptomatic Obstructive Hypertrophic Cardiomyopathy: Results From SEQUOIA-HCM","volume":"13","author":"Coats","year":"2024","journal-title":"J Am Heart Assoc"}],"container-title":["Open Heart"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/openhrt-2025-003192","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,16]],"date-time":"2025-08-16T19:02:01Z","timestamp":1755370921000},"score":1,"resource":{"primary":{"URL":"https:\/\/openheart.bmj.com\/lookup\/doi\/10.1136\/openhrt-2025-003192"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1]]},"references-count":17,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2025,3,28]]},"published-print":{"date-parts":[[2025,1]]}},"alternative-id":["10.1136\/openhrt-2025-003192"],"URL":"https:\/\/doi.org\/10.1136\/openhrt-2025-003192","relation":{},"ISSN":["2053-3624"],"issn-type":[{"value":"2053-3624","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1]]}}}