The patient was a 54-year-old gentleman with sudden chest pain. He suffered from cardiac tamponade and malperfusion of the left carotid artery and the right lower extremity due to acute type A aortic dissection. Rupture of the aortic root and a huge entry from the transverse arch to the proximal descending aorta were found. Extended repairs of valve-sparing root replacement and total arch replacement with frozen elephant trunk were successfully performed. He was discharged without any complications. He was finally diagnosed as having vascular Ehlers-Danlos syndrome by a genetic examination.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC9257385PMC
http://dx.doi.org/10.3400/avd.cr.22-00024DOI Listing

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