Absence of Right Superior Vena Cava and Aortic Annular Hypoplasia in a Patient with Turner's Syndrome

  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.