title: Progressive supra-aortic stenosis in a young adult with the findings of Singleton Merten Syndrome.
abstract: Singleton Merten Syndrome is an autosomal dominant disorder of unknown origin. Patients often present with muscular weakness, failure to thrive, abnormal dentition, glaucoma, psoriatic skin lesions, aortic calcification and musculoskeletal abnormalities. In this case, we present a young girl with a history of aortic root replacement, who had an unusual progressive supra-aortic stenosis managed with urgent surgery during the course of the syndrome. Cardiovascular involvement needs special attention, since it is the major cause of mortality along with rhythm disturbances in the course of Singleton Merten Syndrome.
authors.affiliation: Department of Cardiovascular Surgery, Medipol University, Istanbul, Turkey.
authors.forename: Arda
authors.initials: A
authors.lastname: Ozyuksel
authors.affiliation: Department of Cardiovascular Surgery, Medipol University, Istanbul, Turkey.
authors.forename: Cihangir
authors.initials: C
authors.lastname: Ersoy
authors.affiliation: Department of Cardiovascular Surgery, Medipol University, Istanbul, Turkey.
authors.forename: Emir
authors.initials: E
authors.lastname: Canturk
authors.affiliation: Department of Cardiovascular Surgery, Medipol University, Istanbul, Turkey.
authors.forename: Atif
authors.initials: A
authors.lastname: Akcevin
mesh_terms: D001018:Aortic Diseases / Q000150:complications* / Q000000981:diagnostic imaging; D001024:Aortic Valve Stenosis / Q000000981:diagnostic imaging / Q000209:etiology* / Q000601:surgery*; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D002114:Calcinosis / Q000000981:diagnostic imaging; D002648:Child; D003744:Dental Enamel Hypoplasia / Q000150:complications* / Q000000981:diagnostic imaging; D018450:Disease Progression; D005260:Female; D006801:Humans; D008663:Metacarpus / Q000002:abnormalities* / Q000000981:diagnostic imaging; D009135:Muscular Diseases / Q000150:complications* / Q000000981:diagnostic imaging; D018126:Odontodysplasia / Q000150:complications* / Q000000981:diagnostic imaging; D010024:Osteoporosis / Q000150:complications* / Q000000981:diagnostic imaging; D011859:Radiography; D012008:Recurrence; D013575:Syncope / Q000209:etiology; D061205:Vascular Calcification / Q000150:complications* / Q000000981:diagnostic imaging