{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,18]],"date-time":"2025-11-18T18:19:38Z","timestamp":1763489978812,"version":"3.45.0"},"reference-count":15,"publisher":"Oxford University Press (OUP)","issue":"11","license":[{"start":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T00:00:00Z","timestamp":1761868800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,10,31]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Beh\u00e7et\u2019s syndrome (BS) is a rare, chronic multisystem inflammatory disorder that can lead to severe cardiovascular complications. Among these, aortic pseudoaneurysms are infrequent and associated with high mortality due to their risk of rupture. The diagnosis of BS remains challenging due to its variable clinical presentation and the absence of specific biomarkers, particularly when the initial presentation is atypical.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case summary<\/jats:title>\n                    <jats:p>We present the case of a 32-year-old former professional football player who presented with severe aortic regurgitation with heart failure due to a pseudoaneurysm of the ascending aorta. Initial suspicion of infective endocarditis and aortic dissection was excluded after a clinical, microbiological, and imaging assessment. Emergent Bentall procedure was successfully performed. Subsequently, typical clinical features, including recurrent oral ulcers, pseudofolliculitis, and superficial venous thrombophlebitis, led to the diagnosis of BS. The patient received systemic immunosuppressive therapy combining corticosteroids and infliximab. At 8-month follow-up, echocardiography demonstrated normalization of left ventricular function and no recurrence or new vascular involvement.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Discussion<\/jats:title>\n                    <jats:p>The case highlights the importance of considering BS in the differential diagnosis of young patients presenting with acute aortic disease. Aortic involvement in BS, although rare, can lead to severe complications. To the best of our knowledge, this is the first reported case of a pseudoaneurysm of the aortic root as the initial manifestation of BS. Rapid surgical intervention followed by appropriate medical therapy led to a favourable outcome. Recognizing BS as a potential cause of acute presentations of ascending aortic disease may ensure early and effective management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ehjcr\/ytaf567","type":"journal-article","created":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T13:13:34Z","timestamp":1761830014000},"source":"Crossref","is-referenced-by-count":0,"title":["Severe aortic regurgitation with acute decompensation as initial presentation of Beh\u00e7et\u2019s syndrome: a case report"],"prefix":"10.1093","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-5314-6026","authenticated-orcid":false,"given":"Soha\u00efb","family":"Mansour","sequence":"first","affiliation":[{"name":"Department of Cardiology, CHU Saint-Pierre, Universit\u00e9 Libre de Bruxelles , Rue aux Laines 105, Brussels 1000 ,","place":["Belgium"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9624-9315","authenticated-orcid":false,"given":"Ivan","family":"Dimov","sequence":"additional","affiliation":[{"name":"Department of Cardiology, CHU Saint-Pierre, Universit\u00e9 Libre de Bruxelles , Rue aux Laines 105, Brussels 1000 ,","place":["Belgium"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-5900-3184","authenticated-orcid":false,"given":"Micha\u00ebl","family":"Rietz","sequence":"additional","affiliation":[{"name":"Department of Cardiology, CHU Saint-Pierre, Universit\u00e9 Libre de Bruxelles , Rue aux Laines 105, Brussels 1000 ,","place":["Belgium"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0543-8760","authenticated-orcid":false,"given":"Abraham J","family":"Beun","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, CHU Saint-Pierre, Universit\u00e9 Libre de Bruxelles , Rue aux Laines 105, Brussels 1000 ,","place":["Belgium"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6154-1546","authenticated-orcid":false,"given":"Philippe","family":"Unger","sequence":"additional","affiliation":[{"name":"Department of Cardiology, CHU Saint-Pierre, Universit\u00e9 Libre de Bruxelles , Rue aux Laines 105, Brussels 1000 ,","place":["Belgium"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2025,10,31]]},"reference":[{"key":"2025111813173703000_ytaf567-B1","doi-asserted-by":"crossref","first-page":"640","DOI":"10.1056\/NEJMra2305712","article-title":"Beh\u00e7et\u2019s syndrome","volume":"390","author":"Saadoun","year":"2024","journal-title":"N Engl J Med"},{"key":"2025111813173703000_ytaf567-B2","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1016\/j.berh.2016.08.002","article-title":"Beh\u00e7et\u2019s disease: how to diagnose and treat vascular involvement","volume":"30","author":"Seyahi","year":"2016","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"2025111813173703000_ytaf567-B3","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1038\/s41584-022-00880-7","article-title":"Vascular Beh\u00e7et syndrome: from pathogenesis to treatment","volume":"19","author":"Bettiol","year":"2023","journal-title":"Nat Rev Rheumatol"},{"key":"2025111813173703000_ytaf567-B4","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1080\/1744666X.2016.1205486","article-title":"Beh\u00e7et\u2019s disease: epidemiology, clinical manifestations, and diagnosis","volume":"13","author":"Davatchi","year":"2017","journal-title":"Expert Rev Clin Immunol"},{"key":"2025111813173703000_ytaf567-B5","doi-asserted-by":"crossref","first-page":"1093","DOI":"10.1016\/S0140-6736(23)02629-6","article-title":"Beh\u00e7et\u2019s syndrome","volume":"403","author":"Emmi","year":"2024","journal-title":"Lancet"},{"key":"2025111813173703000_ytaf567-B6","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1007\/s11739-019-02046-y","article-title":"Phenotypes in Beh\u00e7et\u2019s syndrome","volume":"14","author":"Seyahi","year":"2019","journal-title":"Intern Emerg Med"},{"key":"2025111813173703000_ytaf567-B7","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1093\/rheumatology\/kew486","article-title":"Exploring the variability in Beh\u00e7et\u2019s disease prevalence: a meta-analytical approach","volume":"57","author":"Maldini","year":"2018","journal-title":"Rheumatol (Oxford)"},{"key":"2025111813173703000_ytaf567-B8","doi-asserted-by":"crossref","first-page":"523","DOI":"10.1016\/j.rmr.2022.04.010","article-title":"Thoracic manifestations of Beh\u00e7et\u2019s disease [in French]","volume":"39","author":"Ghembaza","year":"2022","journal-title":"Rev Mal Respir"},{"key":"2025111813173703000_ytaf567-B9","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1097\/BOR.0000000000000458","article-title":"A review of current management of vasculo-Beh\u00e7et\u2019s","volume":"30","author":"Merashli","year":"2018","journal-title":"Curr Opin Rheumatol"},{"key":"2025111813173703000_ytaf567-B10","doi-asserted-by":"crossref","first-page":"127","DOI":"10.2147\/JEP.S265645","article-title":"Experimental therapeutic solutions for Beh\u00e7et\u2019s disease","volume":"13","author":"Bozca","year":"2021","journal-title":"J Exp Pharmacol"},{"key":"2025111813173703000_ytaf567-B11","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1186\/1750-1172-9-42","article-title":"Ethnicity and association with disease manifestations and mortality in Beh\u00e7et\u2019s disease","volume":"9","author":"Savey","year":"2014","journal-title":"Orphanet J Rare Dis"},{"key":"2025111813173703000_ytaf567-B12","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1016\/j.cpcardiol.2017.10.004","article-title":"Aortic valve regurgitation: a comprehensive review","volume":"43","author":"Akinseye","year":"2018","journal-title":"Curr Probl Cardiol"},{"key":"2025111813173703000_ytaf567-B13","doi-asserted-by":"crossref","first-page":"EVIDoa2300354","DOI":"10.1056\/EVIDoa2300354","article-title":"Infliximab versus cyclophosphamide for severe Beh\u00e7et\u2019s syndrome","volume":"3","author":"Saadoun","year":"2024","journal-title":"NEJM Evid"},{"key":"2025111813173703000_ytaf567-B14","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1136\/annrheumdis-2018-213225","article-title":"2018 update of the EULAR recommendations for the management of Beh\u00e7et\u2019s syndrome","volume":"77","author":"Hatemi","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"2025111813173703000_ytaf567-B15","doi-asserted-by":"crossref","first-page":"1769","DOI":"10.1007\/s10067-021-06031-9","article-title":"Clinical characteristics and radiographic outcomes of vascular Beh\u00e7et\u2019s disease involving the aorta or its major branches","volume":"41","author":"Lee","year":"2022","journal-title":"Clin Rheumatol"}],"container-title":["European Heart Journal - Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/ehjcr\/advance-article-pdf\/doi\/10.1093\/ehjcr\/ytaf567\/65044513\/ytaf567.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ehjcr\/article-pdf\/9\/11\/ytaf567\/65044513\/ytaf567.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/ehjcr\/article-pdf\/9\/11\/ytaf567\/65044513\/ytaf567.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,18]],"date-time":"2025-11-18T18:17:47Z","timestamp":1763489867000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/ehjcr\/article\/doi\/10.1093\/ehjcr\/ytaf567\/8307102"}},"subtitle":[],"editor":[{"given":"Edoardo","family":"Conte","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]},{"given":"Carlos Nicolas","family":"Perez-Garcia","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]},{"given":"Bruno","family":"Rocha","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]},{"given":"Dimitris","family":"Karelas","sequence":"additional","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2025,10,31]]},"references-count":15,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2025,10,31]]}},"URL":"https:\/\/doi.org\/10.1093\/ehjcr\/ytaf567","relation":{},"ISSN":["2514-2119"],"issn-type":[{"type":"electronic","value":"2514-2119"}],"subject":[],"published-other":{"date-parts":[[2025,11]]},"published":{"date-parts":[[2025,10,31]]},"article-number":"ytaf567"}}