We herein report the performance of a successful, modified Bentall procedure and a total arch replacement for a Stanford type A chronic aortic dissection and a bicuspid aortic valve in Turner's syndrome (TS). The patient was a 45-year-old woman with 45, XO karyotype TS, who had had a history of hypertension since the age of 20. She had also been diagnosed as having a dilatation of the ascending aorta and a bicuspid aortic valve 3 years earlier. The patient became aware of back pain 6 months prior to the current admission, and was diagnosed as having a Stanford type A chronic aortic dissection and a bicuspid aortic valve with mild aortic regurgitation. One of the greatest concerns in TS is the risk of aortic dissection. Regarding the operation, aortic root replacement is one of the options for a bicuspid aortic valve so as to avoid high-risk surgical procedures in TS.
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