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.

Endovascular repair of dissecting thoracic aortic aneurysm in a patient with turner syndrome.

Petrov I, Nedevska M, Chilingirova N, Simeonov P, Kratunkov P, Stoinova V, Nikolov D, Konteva M, Tzarianski G, Tschirkov AI

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 (53x75-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.

Published 17 October 2006 in J Endovasc Ther, 13(5): 693-6.
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