Concerning syndromic TAA in patients diagnosed as vEDS, few post-mortem cases were reported in 2010 [107], and 33 unrelated individuals or families were found to carry COL3A1 splicing mutations or small deletions partially removing splice-junctions sequences [108], and patients developing post-surgical or sudden aortic events are reported [109,110,111]. This evidence concerns the gene COL3A1 and Ehlers-Danlos syndrome, vascular type.