Turner Syndrome Research Today is a free monthly online journal that collates and summarizes the latest research about Turner Syndrome, including details on symptoms, causes, chromosomes, prognosis.

Absence of right superior vena cava and aortic annular hypoplasia in a patient with Turner's syndrome.

Kutay V, Yakut C

Yüzüncü Yil University, Faculty of Medicine, Cardiovascular Surgery Clinic, Van, Turkey. vkutay@yahoo.com

Cardiovascular abnormalities are frequently encountered in patients with Turner's syndrome. These include coarctation of the aorta, bicuspid aortic valve, aortic root dilatation, atrial and ventricular septal defects, but absence of the right superior vena cava (SVC) in visceroatrial situs solitus is extremely rare. We report absence of the right SVC and congenital aortic annular hypoplasia with bicuspid aortic valve stenosis in a patient with Turner's syndrome. Aortic root extension with prosthetic valve replacement was performed and absent right SVC, which was detected incidentally during operation, is confirmed by postoperative venous angiogram.

Published 29 June 2005 in J Card Surg, 20(4): 375-6.
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