{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T08:02:08Z","timestamp":1770883328892,"version":"3.50.1"},"reference-count":5,"publisher":"Oxford University Press (OUP)","issue":"9","license":[{"start":{"date-parts":[[2024,8,13]],"date-time":"2024-08-13T00:00:00Z","timestamp":1723507200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,9,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:sec>\n                  <jats:title>Background<\/jats:title>\n                  <jats:p>Desmoplakin (DSP) cardiomyopathy is a distinct form of cardiomyopathy characterized by frequent left ventricular involvement with extensive fibrosis, high arrhythmic risk, and episodes of acute myocardial injury.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Case summary<\/jats:title>\n                  <jats:p>We are reporting diagnosis and management of a rare case of DSP cardiomyopathy. A patient in his 70s was investigated for mild shortness of breath, recurrent chest infection, and multiple ventricular ectopic. His echocardiogram showed impaired systolic function and found to have 53% ectopic burden with run of non-sustained ventricular tachycardia on 24\u2005h electrocardiogram. Cardiac magnetic resonance imaging showed findings highly suggestive of DSP cardiomyopathy. High-resolution computed tomography chest suggested connective tissue\u2013related interstitial lung disease. The diagnosis of DSP cardiomyopathy was confirmed by genetic testing that demonstrated mutation in DSP protein. The patient had implantable cardioverter-defibrillator implantation for primary prevention.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Discussion<\/jats:title>\n                  <jats:p>Implantable cardioverter-defibrillator implantation should be considered with left ventricular ejection fraction thresholds &amp;gt;35%, particularly in the presence of additional risk factors.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytae419","type":"journal-article","created":{"date-parts":[[2024,8,13]],"date-time":"2024-08-13T18:27:49Z","timestamp":1723573669000},"source":"Crossref","is-referenced-by-count":1,"title":["Desmoplakin cardiomyopathy: case report"],"prefix":"10.1093","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5321-4377","authenticated-orcid":false,"given":"Ayman","family":"Helal","sequence":"first","affiliation":[{"name":"Department of Cardiology, Kettering General Hospital , University Hospitals of Northamptonshire, Rothwell Road, Kettering, NN16 8UZ ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7277-5145","authenticated-orcid":false,"given":"Mohamed","family":"Alama","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Kettering General Hospital , University Hospitals of Northamptonshire, Rothwell Road, Kettering, NN16 8UZ ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wael","family":"Ali","sequence":"additional","affiliation":[{"name":"Department of Radiology, Kettering General Hospital , University Hospitals of Northamptonshire, Rothwell Road, Kettering, NN16 8UZ ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0011-2291","authenticated-orcid":false,"given":"Mohsen","family":"Farooq","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Kettering General Hospital , University Hospitals of Northamptonshire, Rothwell Road, Kettering, NN16 8UZ ,","place":["UK"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2024,8,13]]},"reference":[{"key":"2024093011211524000_ytae419-B1","doi-asserted-by":"crossref","first-page":"2660","DOI":"10.3390\/jcm12072660","article-title":"Desmoplakin cardiomyopathy: comprehensive review of an increasingly recognized entity","volume":"12","author":"Brand\u00e3o","year":"2023","journal-title":"J Clin Med"},{"key":"2024093011211524000_ytae419-B2","doi-asserted-by":"crossref","first-page":"1872","DOI":"10.1161\/CIRCULATIONAHA.119.044934","article-title":"Desmoplakin cardiomyopathy, a fibrotic and inflammatory form of cardiomyopathy distinct from typical dilated or arrhythmogenic right ventricular cardiomyopathy","volume":"141","author":"Smith","year":"2020","journal-title":"Circulation"},{"key":"2024093011211524000_ytae419-B3","doi-asserted-by":"crossref","first-page":"268","DOI":"10.1093\/europace\/euab183","article-title":"Clinical characteristics and risk stratification of desmoplakin cardiomyopathy","volume":"24","author":"Wang","year":"2022","journal-title":"Europace"},{"key":"2024093011211524000_ytae419-B4","doi-asserted-by":"crossref","first-page":"797","DOI":"10.1016\/j.jacc.2023.12.015","article-title":"Phenotypic expression and clinical outcomes in patients with arrhythmogenic cardiomyopathies","volume":"83","author":"Bariani","year":"2024","journal-title":"J Am Coll Cardiol"},{"key":"2024093011211524000_ytae419-B5","doi-asserted-by":"crossref","first-page":"3503","DOI":"10.1093\/eurheartj\/ehad194","article-title":"2023 ESC guidelines for the management of cardiomyopathies: developed by the task force on the management of cardiomyopathies of the European Society of Cardiology (ESC)","volume":"44","author":"Arbelo","year":"2023","journal-title":"Eur Heart J"}],"container-title":["European Heart Journal - 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