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.