{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,27]],"date-time":"2026-10-27T21:37:32Z","timestamp":1793137052840,"version":"4.5.0"},"reference-count":19,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"15","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2024,8,6]]},"abstract":"<jats:sec xml:lang=\"en\">\n                    <jats:title>Background<\/jats:title>\n                    <jats:p xml:lang=\"en\">Patients with obstructive hypertrophic cardiomyopathy have increased symptomatic burden. Mavacamten was recently approved for treatment of obstructive hypertrophic cardiomyopathy based on 2 randomized controlled trials. However, its use under real\u2010world conditions and in diverse populations is under\u2010studied.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Methods and Results<\/jats:title>\n                    <jats:p xml:lang=\"en\">\n                      This was a prospective observational cohort study of patients seen at the Johns Hopkins HCM center and prescribed mavacamten for obstructive hypertrophic cardiomyopathy between July 7, 2022 and January 6, 2024. Patients were followed longitudinally, with serial echocardiography and clinical evaluation as mandated by the risk evaluation and mitigation strategy program. Sixty\u2010six patients received mavacamten (mean age 59\u2009years, 47% male, 29% non\u2010White [Black, Hispanic\/Latino, Asian, Native Hawaiian or Pacific Islander], 47% obese). Before treatment, all patients had New York Heart Association class II (51.5%) or III (48.5%) heart failure symptoms. Initial maximum peak left ventricular outflow tract gradient was 107\u00b146\u2009mm\u2009Hg. Median treatment duration was 9\u2009months. For patients on mavacamten after \u22656 months (n=43), symptoms improved by \u22651 New York Heart Association class in 72% of patients, and peak left ventricular outflow tract gradient decreased by 80\u00b146\u2009mm\u2009Hg, eliminating hemodynamically significant left ventricular outflow tract obstruction in 79.1% of patients. Mavacamten was temporarily discontinued in 3 patients due to left ventricular ejection fraction decrease &lt;50%. There were no medication\u2010related adverse events. Effectiveness and safety were similar between White and non\u2010White patients, but symptomatic relief was attenuated in patients with body\u2010mass index \u226535\u2009kg\/m\n                      <jats:sup>2<\/jats:sup>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec xml:lang=\"en\">\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p xml:lang=\"en\">Mavacamten was effective and safe when used under real\u2010world conditions in a racially diverse population of symptomatic patients with obstructive hypertrophic cardiomyopathy. Patients with comorbid obesity were less likely to experience symptomatic improvement while on mavacamten.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/jaha.123.034069","type":"journal-article","created":{"date-parts":[[2024,7,31]],"date-time":"2024-07-31T07:17:08Z","timestamp":1722410228000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":34,"title":["Eighteen\u2010Month Real\u2010World Experience Using Mavacamten for Treatment of Obstructive Hypertrophic Cardiomyopathy in a Racially Diverse Population"],"prefix":"10.1161","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3884-149X","authenticated-orcid":false,"given":"Diego","family":"Ramonfaur","sequence":"first","affiliation":[{"name":"Johns Hopkins University","place":["Baltimore"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3432-070X","authenticated-orcid":false,"given":"Alessio","family":"Gasperetti","sequence":"additional","affiliation":[{"name":"Johns Hopkins University","place":["Baltimore"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6237-3062","authenticated-orcid":false,"given":"Victoria E.","family":"Blake","sequence":"additional","affiliation":[{"name":"Johns Hopkins University","place":["Baltimore"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bryana","family":"Rivers","sequence":"additional","affiliation":[{"name":"Johns Hopkins University","place":["Baltimore"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ali A.","family":"Kassamali","sequence":"additional","affiliation":[{"name":"Johns Hopkins University","place":["Baltimore"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5730-5433","authenticated-orcid":false,"given":"Edward K.","family":"Kasper","sequence":"additional","affiliation":[{"name":"Johns Hopkins University","place":["Baltimore"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8320-0806","authenticated-orcid":false,"given":"Lili A.","family":"Barouch","sequence":"additional","affiliation":[{"name":"Johns Hopkins University","place":["Baltimore"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4958-5145","authenticated-orcid":false,"given":"Katherine C.","family":"Wu","sequence":"additional","affiliation":[{"name":"Johns Hopkins University","place":["Baltimore"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9966-2703","authenticated-orcid":false,"given":"Jose A.","family":"Madrazo","sequence":"additional","affiliation":[{"name":"Johns Hopkins University","place":["Baltimore"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5589-1216","authenticated-orcid":false,"given":"Richard T.","family":"Carrick","sequence":"additional","affiliation":[{"name":"Johns Hopkins University","place":["Baltimore"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jchf.2018.03.004"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2021.12.002"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.106.644682"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIR.0000000000000938"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehu284"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(20)31792-X"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2022.04.048"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(07)61602-X"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehad194"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1186\/s13063-015-1023-4"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1136\/bmjopen-2022-071473"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1517\/17425255.2012.657626"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.echo.2024.01.009"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1177\/10600280221117812"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.18553\/jmcp.2022.28.3.369"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1161\/CIR.0000000000001250"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.cardfail.2023.11.007"},{"key":"e_1_3_2_19_2","volume-title":"A Long\u2010Term Safety Extension Study of Mavacamten (MYK\u2010461) in Adults With Hypertrophic Cardiomyopathy Who Have Completed the MAVERICK\u2010HCM (MYK\u2010461\u2010006) or EXPLORER\u2010HCM (MYK\u2010461\u2010005) Trials (MAVA\u2010LTE)","author":"Bristol\u2010Myers\u2010Squibb","unstructured":"Bristol\u2010Myers\u2010Squibb . A Long\u2010Term Safety Extension Study of Mavacamten (MYK\u2010461) in Adults With Hypertrophic Cardiomyopathy Who Have Completed the MAVERICK\u2010HCM (MYK\u2010461\u2010006) or EXPLORER\u2010HCM (MYK\u2010461\u2010005) Trials (MAVA\u2010LTE). National Institutes of Health. ClinicalTrials.gov: NCT03723655 (https:\/\/clinicaltrials.gov\/study\/NCT03723655). Start date: 2018\u201010\u20105. Updated: 2024\u201003\u201006. Accessed May 20, 2024"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jchf.2023.09.028"}],"container-title":["Journal of the American Heart Association"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/JAHA.123.034069","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,16]],"date-time":"2025-04-16T23:46:27Z","timestamp":1744847187000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/JAHA.123.034069"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,8,6]]},"references-count":19,"journal-issue":{"issue":"15","published-print":{"date-parts":[[2024,8,6]]}},"alternative-id":["10.1161\/JAHA.123.034069"],"URL":"https:\/\/doi.org\/10.1161\/jaha.123.034069","relation":{},"ISSN":["2047-9980"],"issn-type":[{"value":"2047-9980","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,8,6]]},"assertion":[{"value":"2023-12-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-06-26","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2024-07-31","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e034069"}}