Table 1. Revised Ghent nosology.

Absent family history

Aortic root dilatation Z-score ≥2 AND ectopia lentis

Aortic root dilatation Z-score ≥2 AND FBN1

Aortic root dilatation Z-score ≥2 AND systemic score ≥7 points*

Ectopia lentis AND FBN1 with known aortic root dilatation

Positive family history

Ectopia lentis

A systemic score ≥7 points*

Aortic root dilatation Z-score ≥2 above 20 years old, ≥3 below 20 years old

*, wrist AND thumb sign [3], wrist OR thumb [1], pectus carinatum [2], Pectus excavatum or chest asymmetry [1], hindfoot deformity [2], plain flat foot [1], spontaneous pneumothorax [2], dural ectasia [2], Protucio acetabulae [2], Scoliosis or thoracolumbar kyphosis [1], reduced elbow extension [1], 3/5 facial features [1], skin striae [1], severe myopia [1], mitral valve prolapse [1], reduced upper segment/lower segment (<0.85 whites, <0.78 blacks AND increased arm span/height >1.05).