Our experience on Bentall procedure in an adult patient with Baraitser-Winter syndrome [Baraitser-Winter sendromlu eriskin bir hastada Bentall prosedürü deneyimimiz]

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. © 2018 All right reserved by the Turkish Society of Cardiovascular Surgery.Baraitser-Winter sendromu, sitoplazmik aktin kodlayan gen defektinin neden olduğu, oldukça nadir bir genetik bozukluktur. Pek çok hasta benzer fenotipe sahip olmasina rağmen, eşlik eden kalp anomalileri çeşitlidir. Iyi tarif edilmiş doğustan kalp hastaliklarinin yani sira, ileriki yillarda altta yatan aktin gen mutasyonuna bağli olarak, bu hastalarda aort anevrizmalari görülebilir. Bu yazida, yeni gelişen aort kapak yetmezliği ve çikan aort anevrizmasi tanisiyla Bentall prosedürü yapilan 26 yaşinda bir erkek olgu sunuldu. © 2018 All right reserved by the Turkish Society of Cardiovascular Surgery.