This is the case of a 30-year-old woman with Turner Syndrome, with Mosaicism, exhibiting a bicuspid aortic valve with aortic stenosis, ascending aortic dilatation, pseudocoarctation of the aorta, left superior vena cava and lusoria subclavian artery. The successful surgical procedure consisted of the replacement of the aortic root and ascending aorta with a composite valved graft.
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