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A case of Turner's syndrome, factor 5 Leiden, and fibrinogen deficiency associated with congenital aortic stenosis complicated by aortic aneurysm and aortic regurgitation
Emre Oteyaka, Leylagül Kaynar, Hasan Erdem Murat Ugurlucan, Halil Turkoglu
Language English Country Czech Republic
Document type Case Reports
- MeSH
- Afibrinogenemia pathology MeSH
- Aortic Valve Stenosis * etiology pathology MeSH
- Diagnostic Imaging methods MeSH
- Adult MeSH
- Cardiac Surgical Procedures * methods MeSH
- Humans MeSH
- Activated Protein C Resistance complications MeSH
- Turner Syndrome * complications MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- Female MeSH
- Publication type
- Case Reports MeSH
The combination of Turner's syndrome, factor 5 Leiden and fibrinogen 1 deficiency is extremely rare. The modified Bentall procedure is a viable option for the management of aortic aneurysms and bicuspid aortic valves in Turner's syndrome to minimise the risks associated with high-risk surgical interventions. We report details of the successful implementation of the Bentall operation in a patient with Turner's syndrome complicated by aortic aneurysm, bicuspid aorta, factor 5 Leiden and fibrinogen deficiency.
Kombinace Turnerova syndromu, leidenské mutace (mutace faktoru V Leiden) a deficitu fibrinogenu 1 se vyskytuje velmi vzácně. Modifikovaná Bentallova operace představuje proveditelný způsob řešení aneurysmat aorty a dvojcípých aortálních chlopní u Turnerova syndromu s cílem omezit na minimum rizika spojená s vysoce rizikovými chirurgickými výkony. V této kazuistice podrobně popisujeme úspěšné provedení Bentallovy operace u pacienta s Turnerovým syndromem komplikovaným aneurysmatem aorty, dvojcípou aortální chlopní, mutací faktoru V Leiden a deficitem fi brinogenu 1.
Department of Cardiovascular Surgery Faculty of Medicine Biruni University Istanbul Turkey
Department of Cardiovascular Surgery Faculty of Medicine Istanbul Medipol University Istanbul Turkey
Department of Hematology Faculty of Medicine Istanbul Medipol University Istanbul Turkey
References provided by Crossref.org
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