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A 57-year-old female with Loeys-Dietz syndrome type 3 and MYH11 gene mutation underwent unexpected intraoperative ascending aortic dissection and subsequent rapid progression of the aortic arch dissecting aneurysm. Intra-surgical contingency plans with regard to aortic dissection and aneurysm should be considered for Loeys-Dietz syndrome, especially with comorbid mutations.

Surgical Management of Intraoperative Aortic Dissection in Type 3 Loeys-Dietz Syndrome with MYH11 Co-Mutation