{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,28]],"date-time":"2026-03-28T00:53:36Z","timestamp":1774659216886,"version":"3.50.1"},"reference-count":6,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2021,5,24]],"date-time":"2021-05-24T00:00:00Z","timestamp":1621814400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2021,5,24]],"date-time":"2021-05-24T00:00:00Z","timestamp":1621814400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Cardiothorac Surg"],"published-print":{"date-parts":[[2021,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>The Maroteaux-Lamy syndrome (Mucopolysaccharidosis type VI) is a rare, inherited metabolic disease that results in progressive tissue accumulation of dermatan-sulfated glycosaminoglycans and inflammatory consequences that almost always affects the heart valves. From the anesthesia point of view, managing the airway and ventilation might be a serious challenge due to specific features of the syndrome. Additionally, it is more than probable that the surgical team will perform a non-straightforward procedure.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Case presentation<\/jats:title>\n                <jats:p>A 42-year-old male with Maroteaux-Lamy syndrome was referred to our department with shortness of breath, due to severe aortic stenosis, and at least moderate mitral valve regurgitation.<\/jats:p>\n                <jats:p>The patient was initially scheduled for aortic valve replacement. After multiple attempts with video assisted laryngoscopy, the endotracheal intubation was achieved with the aid of fiberoptic bronchoscopy, while the ventilation succeeded only with laryngeal mask. The somatic features of the syndrome that made the anesthesia induction extremely difficult, also affected the surgical procedure. Suboptimal exposure of the mitral valve, patch enlargement of the aortic root to host the bigger possible prosthesis, and the hard decision to replace the mitral valve even with a marginal indication were the intraoperative challenges for the surgical team.<\/jats:p>\n                <jats:p>Finally, the patient underwent a successful double valve replacement with aortic root enlargement and 18\u2009months postoperatively remains improved.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>Patients with Maroteaux-Lamy syndrome represent a challenge for both anesthesiologists and cardiac surgeons. The whole team should be well prepared to deal with difficulties in airway management, ventilation and surgical valve exposure. The cardiac surgeon should be ready to offer additional procedures and even replace \u201cprematurely\u201d a moderately diseased valve in order to avoid a dangerous reoperation. The limited knowledge on the natural history of the Maroteaux-Lamy syndrome valvulopathy and the difficulties in anesthesia induction support this approach.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13019-021-01530-x","type":"journal-article","created":{"date-parts":[[2021,5,24]],"date-time":"2021-05-24T10:02:43Z","timestamp":1621850563000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Double valve replacement in a patient with Maroteaux \u2013 Lamy syndrome\u00a0as\u00a0an ultimate team challenge"],"prefix":"10.1186","volume":"16","author":[{"given":"Alexandros Agron","family":"Demis","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stella","family":"Oikonomidou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fotios","family":"Daglis","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Spyridon","family":"Polymenakos","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthew","family":"Panagiotou","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2021,5,24]]},"reference":[{"issue":"12","key":"1530_CR1","doi-asserted-by":"publisher","first-page":"1788","DOI":"10.1002\/humu.23613","volume":"39","author":"R Tomanin","year":"2018","unstructured":"Tomanin R, Karageorgos L, Zanetti A, al-Sayed M, Bailey M, Miller N, et al. Mucopolysaccharidosis type VI and molecular analysis: review and classification of published variants in the ARSB gene. Hum Mutat. 2018;39(12):1788\u2013802. https:\/\/doi.org\/10.1002\/humu.23613 Epub 2018 Sep 17.","journal-title":"Hum Mutat"},{"issue":"6","key":"1530_CR2","doi-asserted-by":"publisher","first-page":"1183","DOI":"10.1007\/s10545-011-9359-8","volume":"34","author":"E Braulin","year":"2011","unstructured":"Braulin E, Harmatz P, Scarpa M, et al. Cardiac disease in patients with mucopolysaccharidosis: presentation, diagnosis and management. J Inherit Met Dis. 2011;34(6):1183\u201397. https:\/\/doi.org\/10.1007\/s10545-011-9359-8. Epub 2011 Jul 9.","journal-title":"J Inherit Met Dis"},{"issue":"1","key":"1530_CR3","doi-asserted-by":"publisher","first-page":"188","DOI":"10.1161\/01.cir.85.1.188","volume":"85","author":"CC Tan","year":"1992","unstructured":"Tan CC, Schaff HV, Miller FA, et al. Valvular heart disease in four patients with Maroteaux \u2013 Lamy syndrome. Circulation. 1992;85(1):188\u201395. https:\/\/doi.org\/10.1161\/01.cir.85.1.188.","journal-title":"Circulation"},{"key":"1530_CR4","doi-asserted-by":"publisher","unstructured":"Bell DJW, He C, Pauli JL, Naidoo R. Maroteaux-Lamy syndrome: a rare and challenging case of mitral valve replacement Asian. Cardiovasc Thorac Ann, 2016. 2018;26(7, Oct 18):560\u20132. https:\/\/doi.org\/10.1177\/0218492316675533.Epub.","DOI":"10.1177\/0218492316675533.Epub"},{"issue":"1","key":"1530_CR5","doi-asserted-by":"publisher","first-page":"e23","DOI":"10.1016\/j.athoracsur.2015.11.062","volume":"102","author":"S Torre","year":"2016","unstructured":"Torre S, Scarpelli M, Salviati A, Buffone E, Faggian G, Luciani GB. Aortic and mitral valve involvement in Marroteaux-Lamy syndrome VI: surgical implications in the enzyme replacement therapy era. Ann Thorac Surg. 2016;102(1):e23\u20135. https:\/\/doi.org\/10.1016\/j.athoracsur.2015.11.062.","journal-title":"Ann Thorac Surg"},{"issue":"Suppl2","key":"1530_CR6","doi-asserted-by":"publisher","first-page":"120","DOI":"10.1186\/s13052-018-0562-1","volume":"44","author":"D Concolino","year":"2018","unstructured":"Concolino D, Deodato F, Parini R. Enzyme replacement therapy: efficacy and limitations. Ital J Pediatr. 2018;44(Suppl2):120. https:\/\/doi.org\/10.1186\/s13052-018-0562-1.","journal-title":"Ital J Pediatr"}],"container-title":["Journal of Cardiothoracic Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13019-021-01530-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s13019-021-01530-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s13019-021-01530-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,5,24]],"date-time":"2021-05-24T10:03:32Z","timestamp":1621850612000},"score":1,"resource":{"primary":{"URL":"https:\/\/cardiothoracicsurgery.biomedcentral.com\/articles\/10.1186\/s13019-021-01530-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,5,24]]},"references-count":6,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2021,12]]}},"alternative-id":["1530"],"URL":"https:\/\/doi.org\/10.1186\/s13019-021-01530-x","relation":{},"ISSN":["1749-8090"],"issn-type":[{"value":"1749-8090","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,5,24]]},"assertion":[{"value":"30 December 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"7 May 2021","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"24 May 2021","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Approval was obtained from the Athens Medical Center Ethics Committee with reference number: KM140721, which confirms that the study was performed in accordance with the Declaration of Helsinki and hasn\u2019t been granted an exemption from requiring ethics approval.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"The written patient\u2019s consent for treatment, participation and publication was obtained.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare that they have no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"141"}}