{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T03:07:49Z","timestamp":1761102469632,"version":"3.37.3"},"reference-count":5,"publisher":"Oxford University Press (OUP)","issue":"5","license":[{"start":{"date-parts":[[2021,6,26]],"date-time":"2021-06-26T00:00:00Z","timestamp":1624665600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2021,10,29]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Hunter syndrome is a rare disease leading to glycosaminoglycan accumulation in tissues. Multiple organs are involved, but prognosis is mainly conditioned by cardiac and respiratory failures. Cardiac valvular impairment is quite common but aortic root dilatation is rarely described. This article covers a case of surgical root replacement due to aortic valve insufficiency and aortic root dilatation documented with magnetic resonance and computed tomography angiographies. Anatomic pathology reported both aortic valve and aorta with mucoid overload and elastic fibre depletion. These patients do have a risk of aortic root dilatation, which justifies periodic monitoring. Diagnosis must be made using indexed measures.<\/jats:p>","DOI":"10.1093\/icvts\/ivab171","type":"journal-article","created":{"date-parts":[[2021,5,24]],"date-time":"2021-05-24T11:09:33Z","timestamp":1621854573000},"page":"819-821","source":"Crossref","is-referenced-by-count":2,"title":["Surgical management of an aortic root dilatation in a patient suffering from Hunter syndrome"],"prefix":"10.1093","volume":"33","author":[{"given":"Bastien","family":"Poitier","sequence":"first","affiliation":[{"name":"Cardiac Surgery Department, AH-HP, Georges Pompidou European Hospital, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mourad","family":"Amrane","sequence":"additional","affiliation":[{"name":"Cardiac Surgery Department, AH-HP, Georges Pompidou European Hospital, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2350-2914","authenticated-orcid":false,"given":"Patrick","family":"Bruneval","sequence":"additional","affiliation":[{"name":"Department of Pathology Anatomy, AH-HP, Georges Pompidou European Hospital, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul","family":"Achouh","sequence":"additional","affiliation":[{"name":"Cardiac Surgery Department, AH-HP, Georges Pompidou European Hospital, Paris, France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2021,6,26]]},"reference":[{"key":"2022032109304881000_ivab171-B1","doi-asserted-by":"crossref","first-page":"15786","DOI":"10.1038\/ncomms15786","article-title":"Insights into Hunter syndrome from the structure of iduronate-2-sulfatase","volume":"8","author":"Demydchuk","year":"2017","journal-title":"Nat Commun"},{"key":"2022032109304881000_ivab171-B2","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1186\/s13023-015-0259-0","article-title":"Diagnosis, quality of life, and treatment of patients with Hunter syndrome in the French healthcare system: a retrospective observational study","volume":"10","author":"Guffon","year":"2015","journal-title":"Orphanet J Rare Dis"},{"key":"2022032109304881000_ivab171-B3","doi-asserted-by":"crossref","DOI":"10.3390\/ijms21041258","article-title":"Mucopolysaccharidosis type II: one hundred years of research, diagnosis, and treatment","volume":"21","author":"D\u2019Avanzo","year":"2020","journal-title":"Int J Mol Sci"},{"key":"2022032109304881000_ivab171-B4","doi-asserted-by":"crossref","DOI":"10.3390\/ijms17122004","article-title":"Aortic root dilatation in mucopolysaccharidosis I-VII","volume":"17","author":"Bolourchi","year":"2016","journal-title":"Int J Mol Sci"},{"key":"2022032109304881000_ivab171-B5","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1161\/CIRCULATIONAHA.106.685487","article-title":"Aortic dilatation and dissection in Turner syndrome","volume":"116","author":"Matura","year":"2007","journal-title":"Circulation"}],"container-title":["Interactive CardioVascular and Thoracic Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/icvts\/article-pdf\/33\/5\/819\/42958447\/ivab171.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/icvts\/article-pdf\/33\/5\/819\/42958447\/ivab171.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,3,21]],"date-time":"2022-03-21T09:31:03Z","timestamp":1647855063000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/icvts\/article\/33\/5\/819\/6309786"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,6,26]]},"references-count":5,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2021,6,26]]},"published-print":{"date-parts":[[2021,10,29]]}},"URL":"https:\/\/doi.org\/10.1093\/icvts\/ivab171","relation":{},"ISSN":["1569-9285"],"issn-type":[{"type":"electronic","value":"1569-9285"}],"subject":[],"published-other":{"date-parts":[[2021,11,1]]},"published":{"date-parts":[[2021,6,26]]}}}