{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,11]],"date-time":"2026-04-11T17:50:37Z","timestamp":1775929837144,"version":"3.50.1"},"reference-count":30,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2023,1,11]],"date-time":"2023-01-11T00:00:00Z","timestamp":1673395200000},"content-version":"vor","delay-in-days":15,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Motyl Graduate Studentship"},{"DOI":"10.13039\/501100000190","name":"University of Alberta","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100000190","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,12,27]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Heart disease is an under-recognized cause of morbidity and mortality in patients with Emery-Dreifuss muscular dystrophy (EDMD). Arrhythmias and conduction delays are highly prevalent and given the rarity of this disease the patient care process remains poorly defined.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary<\/jats:title>\n                  <jats:p>This study closely followed four adult patients from the Neuromuscular Multidisciplinary Clinic (Alberta, Canada) that presented with X-linked recessive EDMD. Patients were assessed and managed on a case-by-case basis. Clinical status and cardiac function were assessed through clinical history, physical examination, and investigations (12-lead electrocardiogram, 24\u2005hour Holter monitor, transthoracic echocardiogram, and plasma biomarkers). Conduction disease, requiring permanent pacemaker, was prevalent in all patients. With appropriate medical therapy over a median follow-up period five years the cardiac status was shown to have stabilized in all these patients.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>We demonstrate the presentation of arrhythmias, conduction abnormalities, and chamber dilation in adult patients with X-linked EDMD. Cardiac medications and pacemaker therapy are shown to prevent adverse outcomes from these complications. Patients with EDMD are expected to develop heart disease early and prior to the development of an overt neuromuscular phenotype. These patients should be closely monitored in a multidisciplinary setting for effective management to improve their clinical outcomes.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytad013","type":"journal-article","created":{"date-parts":[[2023,1,11]],"date-time":"2023-01-11T18:06:48Z","timestamp":1673460408000},"source":"Crossref","is-referenced-by-count":6,"title":["Cardiac manifestations and clinical management of X-linked Emery-Dreifuss muscular dystrophy: a case series"],"prefix":"10.1093","volume":"7","author":[{"given":"Niharika","family":"Kashyap","sequence":"first","affiliation":[{"name":"Division of Cardiology, Department of Medicine, University of Alberta , 8440 112 Street NW, Edmonton, Alberta T6G 2R7 , Canada"},{"name":"Mazankowski Alberta Heart Institute, University of Alberta , 11220 83 Avenue NW, Edmonton, Alberta T6G 2J2 , Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2247-4907","authenticated-orcid":false,"given":"Anish","family":"Nikhanj","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine, University of Alberta , 8440 112 Street NW, Edmonton, Alberta T6G 2R7 , Canada"},{"name":"Mazankowski Alberta Heart Institute, University of Alberta , 11220 83 Avenue NW, Edmonton, Alberta T6G 2J2 , Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luke R","family":"Gagnon","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine, University of Alberta , 8440 112 Street NW, Edmonton, Alberta T6G 2R7 , Canada"},{"name":"Mazankowski Alberta Heart Institute, University of Alberta , 11220 83 Avenue NW, Edmonton, Alberta T6G 2J2 , Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Basel","family":"Moukaskas","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine, University of Alberta , 8440 112 Street NW, Edmonton, Alberta T6G 2R7 , Canada"},{"name":"Mazankowski Alberta Heart Institute, University of Alberta , 11220 83 Avenue NW, Edmonton, Alberta T6G 2J2 , Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zaeem A","family":"Siddiqi","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Medicine, University of Alberta , 8440 112 Street NW, Edmonton, Alberta T6G 2R7 , Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9154-9028","authenticated-orcid":false,"given":"Gavin Y","family":"Oudit","sequence":"additional","affiliation":[{"name":"Division of Cardiology, Department of Medicine, University of Alberta , 8440 112 Street NW, Edmonton, Alberta T6G 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