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.