(Ghent-2 Pos, revised Ghent Criteria positive; Ghent-2 Neg, revised Ghent Criteria negative; FBN1 Pos, FBN1 mutation positive; FBN1 Neg, FBN1 mutation negative; SV, dilatation of sinus of valsalvae; PA, dilatation of pulmonary artery; MVP, mitral valve prolapse; TVP, tricuspid valve prolapse; 3Skel, at least 3 skeletal features of the systemic score of the revised Ghent Criteria; EL, ectopia lentis; FH, family history). This evidence concerns the gene FBN1 and isolated ectopia lentis.