ISSN : 1301-5680
e-ISSN : 2149-8156
TURKISH JOURNAL OF
THORACIC AND
CARDIOVASCULAR SURGERY
Turkish Journal of Thoracic and Cardiovascular Surgery     
Our experience on Bentall procedure in an adult patient with Baraitser-Winter syndrome
Gökmen Akkaya1, Çağatay Bilen1, Osman Nuri Tuncer1, Mehmet Fatih Ayık1, Yüksel Atay1
1Department of Pediatric Cardiovascular Surgery, Ege University Faculty of Medicine, İzmir, Turkey
DOI : 10.5606/tgkdc.dergisi.2018.15758
Baraitser-Winter syndrome is a very rare genetic disorder caused by cytoplasmic actin-encoding genes defects. Although most patients have similar phenotype, concomitant cardiac anomalies widely vary. In addition to well-described congenital heart diseases, aortic aneurysms occur due to underlying actin gene mutation in these patients in the further years. Herein, we present a 26-year-old male case who underwent Bentall procedure with the diagnosis of new-onset aortic valve regurgitation and an ascending aorta aneurysm.
Keywords : Adulthood; ascending aortic aneurysm; Baraitser-Winter syndrome; Bentall procedure
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