Purpose: To report a rare case of dissecting thoracic aortic aneurysm in a young patient with Turner syndrome owing to complete or partial monosomy of the X chromosome.Case Report: A 22-year-old patient with Turner syndrome presented with a 2-month history of voice loss and dysphagia. Multislice computed tomography (MSCT) disclosed a large (53×75-mm) aneurysm with focal dissection affecting the distal part of the aortic arch and the proximal descending aorta, partially involving the left subclavian artery. A TAG endoprosthesis was implanted without complications. MSCT scans at 3 and 6 months after the procedure showed good position and patency of the stent-graft, with total exclusion and shrinkage of the aneurysm. After 1 year of follow-up, she is doing well, without voice disturbances or dysphagia.
Conclusion: Although cardiovascular malformations are common in patients with Turner syndrome, dissecting thoracic aortic aneurysm is unusual. Stent-graft repair would appear to be feasible in this situation, but long-term implantation in young patients has not been explored.
