DISCUSSION
vEDS is a hereditary syndrome caused by impaired metabolism of fibrous collagen. Because
of its vascular fragility, surgical outcomes are generally poor [3]. VSRR was challenging
because it is more technically demanding [4]. Particularly for vEDS, there are only a few reports about emergency case with AAAD [5]. VSRR was however attempted, because of relatively young age and no significant deformities of the aortic valve, and of the surgeon’s (HO) experiences over 200 VSRRs. No adventitia was preserved at the commissure between the right- and non-coronary cusps. Careful attachment of such a destroyed and fragile commissure to the graft was required. Apart from remodeling procedures, the commissure posts are inserted in the graft and attached to its inside, which is one of the advantages of reimplantation techniques. During surgery, it was suspected that the patient had a sort of connective tissue disease because of his tissue fragility. Finally, it was revealed as vEDS. This is the first report on a vEDS patient having VSRR and TAR with FET for AAAD successfully performed. In vEDS, severe follow-up of the anastomosis is necessary because of the fragility of the tissue.