{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T17:24:02Z","timestamp":1789665842898,"version":"4.0.1"},"reference-count":49,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2023,10,18]],"date-time":"2023-10-18T00:00:00Z","timestamp":1697587200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000030","name":"Centers for Disease Control and Prevention","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000030","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Birth Defects Research"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Epidemiological support for prophylactic treatment of left ventricular dysfunction (LVD) in Duchenne muscular dystrophy is limited. We used retrospective, population\u2010based surveillance data from the Muscular Dystrophy Surveillance, Tracking and Research Network to evaluate whether prophylaxis delays LVD onset.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We analyzed 455 males born during 1982\u20132009. Age at first abnormal echocardiogram (ejection fraction &lt;55% or shortening fraction &lt;28%) determined LVD onset. Prophylaxis was defined as cardiac medication use at least 1\u2009year prior to LVD. Corticosteroid use was also coded. Kaplan\u2013Meier curve estimation and Cox Proportional Hazard modeling with time\u2010varying covariates describe associations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>LVD was identified among 40.7%; average onset age was 14.2\u2009years. Prophylaxis was identified for 20.2% and corticosteroids for 57.4%. Prophylaxis showed delayed LVD onset (<jats:italic>p<\/jats:italic>\u2009&lt;\u2009.001) and lower hazard of dysfunction (adjusted hazard ratio [aHR]\u2009=\u20090.39, 95%CL\u2009=\u20090.22, 0.65) compared to untreated. Compared to no treatment, continuous corticosteroids only (aHR\u2009=\u20091.01, 95%CL\u2009=\u20090.66, 1.53) and prophylaxis only (aHR\u2009=\u20090.67, 95%CL\u2009=\u20090.25, 1.50) were not cardioprotective, but prophylaxis plus continuous corticosteroids were associated with lower hazard of dysfunction (aHR\u2009=\u20090.37, 95%CL\u2009=\u20090.15, 0.80).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Proactive cardiac treatment and monitoring are critical aspects of managing Duchenne muscular dystrophy. Consistent with clinical care guidelines, this study supports clinical benefit from cardiac medications initiated prior to documented LVD and suggests further benefit when combined with corticosteroids.<\/jats:p><\/jats:sec>","DOI":"10.1002\/bdr2.2260","type":"journal-article","created":{"date-parts":[[2023,10,18]],"date-time":"2023-10-18T11:59:31Z","timestamp":1697630371000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Prophylactic use of cardiac medications for delay of left ventricular dysfunction in Duchenne muscular dystrophy"],"prefix":"10.1002","volume":"116","author":[{"given":"Kristin M.","family":"Conway","sequence":"first","affiliation":[{"name":"Department of Epidemiology College of Public Health, The University of Iowa  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3099-4520","authenticated-orcid":false,"given":"Shiny","family":"Thomas","sequence":"additional","affiliation":[{"name":"New York State Department of Health  Albany New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma","family":"Ciafaloni","sequence":"additional","affiliation":[{"name":"Department of Neurology School of Medicine and Dentistry, University of Rochester Medical Center  Rochester New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rabia S.","family":"Khan","sequence":"additional","affiliation":[{"name":"Department of Pediatrics UCLA Health Sciences  Los Angeles California USA"},{"name":"Department of Pediatrics Roy J and Lucille A Carver College of Medicine, The University of Iowa  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joshua R.","family":"Mann","sequence":"additional","affiliation":[{"name":"Department of Preventive Medicine School of Medicine and John D. 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