Extended Aortic Repair for Acute Type A Aortic Dissection with Rupture and Malperfusion Complicated with Ehlers-Danlos Syndrome.

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.