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

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AKKAYA G. , BİLEN Ç. , TUNCER O. N. , AYIK M. F. , ATAY Y.

TURK GOGUS KALP DAMAR CERRAHISI DERGISI-TURKISH JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY, cilt.26, ss.639-641, 2018 (SCI İndekslerine Giren Dergi) identifier identifier

  • Cilt numarası: 26 Konu: 4
  • Basım Tarihi: 2018
  • Doi Numarası: 10.5606/tgkdc.dergisi.2018.15758
  • Dergi Adı: TURK GOGUS KALP DAMAR CERRAHISI DERGISI-TURKISH JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
  • Sayfa Sayıları: ss.639-641

Özet

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.