Case presentation
The patient was a 54-year-old gentleman who suffered from cardiac tamponade, 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 located from the transverse arch to the proximal descending aorta were found. There were no significant deformities at aortic valve, so we tried extended repairs of valve sparing root replacement (VSRR) and total arch replacement (TAR) with frozen elephant trunk (FET) and were successfully performed. He was finally diagnosed as vascular Ehlers-Danlos syndrome (vEDS) by genetic examination.