A case of Turner's syndrome, factor 5 Leiden, and fibrinogen deficiency associated with congenital aortic stenosis complicated by aortic aneurysm and aortic regurgitation

The combination of Turner's syndrome, factor 5 Leiden and fi brinogen 1 defi ciency is extremely rare. The modified Bentall procedure is a viable option for the management of aortic aneurysms and bicuspid aortic valves in Turner's syndrome to minimise the risks associated with high -risk surgical interventions. We report details of the successful implementation of the Bentall operation in a patient with Turner's syndrome complicated by aortic aneurysm, bicuspid aorta, factor 5 Leiden and fibrinogen deficiency.