FBN1 and isolated ectopia lentis: Revised Ghent criteria for MFS diagnosis, establishes four scenarios in absence of family history for a proband: Aortic root enlargement (Z-score ≥2.0) plus ectopia lentis, or with a pathogenic FBN1 variant; or with a systemic score ≥7; or with ectopia lentis and FBN1 pathogenic variant.