Aortic dissection and Turner's syndrome

Cardiovascular malformations, especially coarctation of the aorta and bicus pid aortic valve, are common in patients with Turner's syndrome. Only 46 pa tients with aortic dissection and/or rupture have been described. All patie nts had associated aortic dilation or aneurysm. In all cases except three, involvement of the ascending aorta was present, making surgery often impera tive. We describe a rare case of a DeBakey type IIIb aortic dissection (wit hout involvement of the proximal aorta) in a patient with Turner's syndrome mosaicism, The dissection occurred two weeks after a caesarean section bec ause of eclampsia. No aortic dilation or other cardiovascular malformations were found. The distal extension and uncomplicated nature of the dissectio n indicated medical management. After fifteen months of followup, she is cl inically doing well and repeated CT scan shows a stable dissection of the d escending and abdominal aorta without dilation.