Our experience on Bentall procedure in an adult patient with Baraitser-Winter syndrome

Gökmen Akkaya

doi:10.5606/tgkdc.dergisi.2018.15758

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

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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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