{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T15:03:47Z","timestamp":1790521427543,"version":"4.1.0"},"reference-count":30,"publisher":"SAGE Publications","issue":"4","license":[{"start":{"date-parts":[[2021,3,29]],"date-time":"2021-03-29T00:00:00Z","timestamp":1616976000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Neuromuscular Diseases"],"published-print":{"date-parts":[[2021,7,30]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>The latest practice guidelines from the American College of Cardiology\/American Heart Association recommend the prescription of an ACE-i for patients presenting with non-ischemic cardiomyopathy when left ventricular ejection fraction (LVEF) falls below 40%.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective:<\/jats:title>\n                    <jats:p>To determine if the initiation of treatment with an angiotensin-converting enzyme inhibitor (ACE-i) earlier than recommended by practice guidelines issued by professional societies improves the long-term cardiac outcomes of patients presenting with Becker muscular dystrophy (MD) cardiomyopathy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>From a multicenter registry of Becker MD, we selected retrospectively patients presenting between January 1990 and April 2019 with a LVEF \u226540 and \u226449%. We used a propensity score analysis to compare the risk of a) hospitalization for management of heart failure (HF), and b) a decrease in LVEF to &lt;35% in patients who received an ACE-i when LVEF fell below 40% (conventional treatment), versus below 50% (early treatment).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>From the 183 patients entered in our registry, we identified 85 whose LVEF was between 40 and 49%, 51 of whom received early and 34 received conventional ACE-i treatment. Among patients with early versus conventional treatments, 2 (3.9%) versus 4 (11.8%) were hospitalized for management of HF [hazard ratio (HR) 0.151; 95% confidence interval (CI) 0.028 to 0.822; p\u200a=\u200a0.029], and 9 (17.6%) versus 10 (29.4%) had a decrease in LVEF below 35% (HR 0.290; 95% CI 0.121 to 0.694; p\u200a=\u200a0.005).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>The long-term cardiac outcome of patients presenting with Becker MD was significantly better when treatment with ACE-i was introduced after a decrease in LVEF below 50%, instead of below 40% as recommended in the current practice guidelines issued by professional societies.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3233\/jnd-200620","type":"journal-article","created":{"date-parts":[[2021,3,30]],"date-time":"2021-03-30T14:42:52Z","timestamp":1617115372000},"page":"495-502","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":8,"title":["Improved Cardiac Outcomes by Early Treatment with Angiotensin-Converting Enzyme Inhibitors in Becker Muscular Dystrophy"],"prefix":"10.1177","volume":"8","author":[{"given":"Caroline","family":"Stalens","sequence":"first","affiliation":[{"name":"Medical Affairs Department, AFM-T\u00e9l\u00e9thon, Evry, France"},{"name":"INSERM Unit 970, Paris Cardiovascular Research Centre (PARCC), Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leslie","family":"Mott\u00e9","sequence":"additional","affiliation":[{"name":"AP-HP, Cochin Hospital, Cardiology Department, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony","family":"B\u00e9hin","sequence":"additional","affiliation":[{"name":"AP-HP, Piti\u00e9-Salp\u00eatri\u00e8re Hospital, Reference Center for Muscle Diseases Paris-Est, Myology Institute, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rabah","family":"Ben Yaou","sequence":"additional","affiliation":[{"name":"AP-HP, Piti\u00e9-Salp\u00eatri\u00e8re Hospital, Reference Center for Muscle Diseases Paris-Est, Myology Institute, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"France","family":"Leturcq","sequence":"additional","affiliation":[{"name":"AP-HP, Cochin Hospital, Department of Genetics and Molecular Biology, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillaume","family":"Bassez","sequence":"additional","affiliation":[{"name":"AP-HP, Piti\u00e9-Salp\u00eatri\u00e8re Hospital, Reference Center for Muscle Diseases Paris-Est, Myology Institute, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"Lafor\u00eat","sequence":"additional","affiliation":[{"name":"Neurology Department, CHU Paris IdF Ouest\u2013H\u00f4pital Raymond Poincar\u00e9, Garches, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bertrand","family":"Fontaine","sequence":"additional","affiliation":[{"name":"AP-HP, Piti\u00e9-Salp\u00eatri\u00e8re Hospital, Reference Center for Muscle Diseases Paris-Est, Myology Institute, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phane","family":"Ederhy","sequence":"additional","affiliation":[{"name":"AP-HP, Saint Antoine Hospital, Cardiology Department, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marion","family":"Masingue","sequence":"additional","affiliation":[{"name":"AP-HP, Piti\u00e9-Salp\u00eatri\u00e8re Hospital, Reference Center for Muscle Diseases Paris-Est, Myology Institute, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malika","family":"Saadi","sequence":"additional","affiliation":[{"name":"AP-HP, Cochin Hospital, Cardiology Department, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah Leonard","family":"Louis","sequence":"additional","affiliation":[{"name":"AP-HP, Piti\u00e9-Salp\u00eatri\u00e8re Hospital, Reference Center for Muscle Diseases Paris-Est, Myology Institute, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nawal","family":"Berber","sequence":"additional","affiliation":[{"name":"AP-HP, Piti\u00e9-Salp\u00eatri\u00e8re Hospital, Reference Center for Muscle Diseases Paris-Est, Myology Institute, Paris, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tanya","family":"Stojkovic","sequence":"additional","affiliation":[{"name":"AP-HP, Piti\u00e9-Salp\u00eatri\u00e8re Hospital, 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France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2021,3,29]]},"reference":[{"key":"ref001","doi-asserted-by":"publisher","DOI":"10.1007\/BF00858726"},{"key":"ref002","doi-asserted-by":"publisher","DOI":"10.1007\/s004150050163"},{"key":"ref003","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/16.23.11141"},{"issue":"1","key":"ref004","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1093\/brain\/117.1.1-a","volume":"117","author":"Comi GP","year":"1994","journal-title":"Brain J Neurol f\u00e9vr"},{"issue":"4","key":"ref005","first-page":"498","volume":"45","author":"Koenig M","year":"1989","journal-title":"Am J Hum Genet oct."},{"key":"ref006","unstructured":"EmeryAEH. 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