{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T15:44:30Z","timestamp":1789055070672,"version":"build-2803163510"},"reference-count":44,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,1,24]],"date-time":"2025-01-24T00:00:00Z","timestamp":1737676800000},"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","award":["U01 DD001248"],"award-info":[{"award-number":["U01 DD001248"]}],"id":[{"id":"10.13039\/100000030","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>Prophylactic treatment of left ventricular dysfunction (LVD) in Duchenne muscular dystrophy (DMD) delays onset of LVD, but there is limited data showing impact on survival. Our aim was to describe survival among treated and untreated individuals with DMD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Retrospective, population\u2010based surveillance data from the Muscular Dystrophy Surveillance, Tracking and Research Network (MD STAR<jats:italic>net<\/jats:italic>) were used. We analyzed 327 males with DMD born between 1982 and 2009 who were at least 6\u2009years old at the last visit and who initiated cardiac prophylactic medication before age 14\u2009years. Death status was ascertained through vital record linkages and medical record review. Prophylaxis was defined as cardiac medication use at least 1 year before LVD onset (ejection fraction &lt;\u200955% or shortening fraction &lt;\u200928%). Age at first visit, corticosteroid use, scoliosis surgery, initiation of noninvasive ventilation, and loss of ambulation were also coded. Cox Proportional Hazard modeling with time\u2010varying covariates describes associations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Prophylactic cardiac treatment was documented for 27.7% (<jats:italic>n<\/jats:italic>\u2009=\u200990); corticosteroids were used by 60.9% (<jats:italic>n<\/jats:italic>\u2009=\u2009157). Adjusting for age at first visit and MD STAR<jats:italic>net<\/jats:italic> site, prophylactic treatment was associated with a 54% lower hazard of death (HR\u2009=\u20090.46, 95% CI\u2009=\u20090.22\u20130.93) compared to no prophylaxis. Adjusting for selected clinical covariates did not appreciably change the estimate (HR\u2009=\u20090.46, 95% CI\u2009=\u20090.22\u20130.99).<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Initiation of cardiac medication when left ventricular function is normal was associated with prolonged survival in this study of males with DMD. Only one\u2010quarter of individuals received this treatment, however, indicating a topic of focus for improving care.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.28353","type":"journal-article","created":{"date-parts":[[2025,1,24]],"date-time":"2025-01-24T08:24:35Z","timestamp":1737707075000},"page":"574-582","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Prophylactic Use of Cardiac Medications and Survival in Duchenne Muscular Dystrophy"],"prefix":"10.1002","volume":"71","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8633-966X","authenticated-orcid":false,"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":"Tahereh","family":"Neyaz","sequence":"additional","affiliation":[{"name":"Department of Epidemiology, College of Public Health The University of Iowa  Iowa City Iowa 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":"Joshua R.","family":"Mann","sequence":"additional","affiliation":[{"name":"Department of Preventive Medicine, School of Medicine and John D. Bower School of Population Health University of Mississippi Medical Center  Jackson Mississippi USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michelle","family":"Staron\u2010Ehlinger","sequence":"additional","affiliation":[{"name":"Stead Family 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":"Gary S.","family":"Beasley","sequence":"additional","affiliation":[{"name":"Stead Family 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"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5393-9984","authenticated-orcid":false,"given":"Paul A.","family":"Romitti","sequence":"additional","affiliation":[{"name":"Department of Epidemiology, College of Public Health The University of Iowa  Iowa City Iowa USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine D.","family":"Mathews","sequence":"additional","affiliation":[{"name":"Stead Family 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"}]},{"name":"the Muscular Dystrophy Surveillance, Tracking and Research Network (MD STARnet)","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,1,24]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00246-020-02309-y"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1161\/JAHA.117.006340"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23215"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(17)32160-8"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2022.04.008"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurheartj\/ehab054"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/openhrt-2018-000783"},{"key":"e_1_2_10_9_1","first-page":"1","article-title":"Prophylactic Use of Cardiac Medications for Delay of Left Ventricular Dysfunction in Duchenne Muscular Dystrophy","volume":"116","author":"Conway K. 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