{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,6]],"date-time":"2026-06-06T01:58:05Z","timestamp":1780711085064,"version":"3.54.1"},"reference-count":34,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2021,6,11]],"date-time":"2021-06-11T00:00:00Z","timestamp":1623369600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>The DMD Care Considerations Working Group Guidelines 2010 recommended treating cardiac dystrophinopathy with angiotensin\u2010converting enzyme\u2010inhibitor (ACEi) and beta\u2010blocker (BB) therapy to prevent the progressive decline in left ventricular function expected from earlier, natural history studies. The aim of this research was to audit change in measures of left ventricular function over 8\u2009years to 4\u2009years before and 4\u2009years after deploying an ACEi\/BB combination systematically at a dedicated \u201ccardiology\u2010muscle\u201d clinic.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This is an institutionally registered, retrospective, case\u2010file\u2013based audit of serial echocardiographic measures of left ventricular fractional shortening accumulated over the period 1995 to 2015.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Data from 104 genetically confirmed Duchenne muscular dystrophy (DMD) patients, aged 22.2\u2009\u00b1\u20095.3\u2009years at data censure, were included. Mean age at first detection of left ventricular dysfunction was 15.1\u00a0\u00b1\u20094.2\u2009years, but older in those on maintenance steroid therapy (16.8\u00a0\u00b1\u20094.2 vs 14.5\u00a0\u00b1\u20094.1\u2009years; <jats:italic>P<\/jats:italic>\u00a0=\u2009.04). Group mean fractional shortening fell by 1.5%\/year over the 4\u2009years before therapy, but this decreased to 0.9%\/year over the first 4\u2009years after starting therapy. Analysis of limited left ventricular ejection fraction measures showed similar but nonsignificant changes. Neither age at detection of left ventricular dysfunction nor fractional shortening percent at time of therapy initiation affected the beneficial response.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>The results support the international DMD recommendations of the time. This combination of cardiac medications helps stabilize heart function. For the best long\u2010term effects, therapy needs to be initiated no later than on first detection left ventricular impairment.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27336","type":"journal-article","created":{"date-parts":[[2021,6,3]],"date-time":"2021-06-03T20:57:55Z","timestamp":1622753875000},"page":"163-171","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Effects of cardiac medications on ventricular function in patients with Duchenne muscular dystrophy\u2013related cardiomyopathy"],"prefix":"10.1002","volume":"64","author":[{"given":"Alexander P.","family":"Murphy","sequence":"first","affiliation":[{"name":"The John Walton Muscular Dystrophy Research Centre, Institute of Translational and Clinical Research Newcastle University  Newcastle upon Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Johnson","sequence":"additional","affiliation":[{"name":"Department of Cardiology Freeman Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust  Newcastle upon Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Volker","family":"Straub","sequence":"additional","affiliation":[{"name":"The John Walton Muscular Dystrophy Research Centre, Institute of Translational and Clinical Research Newcastle University  Newcastle upon Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alison","family":"Heads\u2010Baister","sequence":"additional","affiliation":[{"name":"Department of Cardiology Freeman Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust  Newcastle upon Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen","family":"Lord","sequence":"additional","affiliation":[{"name":"Department of Cardiology Freeman Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust  Newcastle upon Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John P.","family":"Bourke","sequence":"additional","affiliation":[{"name":"Department of Cardiology Freeman Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust  Newcastle upon Tyne UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,6,11]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(18)30024-3"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(02)00140-2"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2016.11.012"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2012.12.008"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2020.04.043"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1186\/1532-429X-13-60"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2016.02.081"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ppul.23215"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/ejhf.592"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCHEARTFAILURE.116.003529"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0960-8966(02)00213-4"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(09)70272-8"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(18)30025-5"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1093\/eurjhf\/hfs105"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(98)11181-9"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(97)80008-6"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jjcc.2008.08.013"},{"issue":"10","key":"e_1_2_9_19_1","first-page":"CD009068","article-title":"Interventions for preventing and treating cardiac complications in Duchenne and Becker muscular dystrophy and X\u2010linked dilated cardiomyopathy","volume":"10","author":"Bourke JP","year":"2018","journal-title":"Cochrane Database Syst Rev"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1530\/ERP-14-0079"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1530\/ERP-14-0003"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/CND.0000000000000204"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jacc.2004.09.078"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.102.22.2700"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(14)70318-7"},{"key":"e_1_2_9_26_1","first-page":"117","article-title":"Improved survival in patients with scoliosis secondary to Duchenne muscular dystrophy. 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