Fig. 4: Risk chart for genetic counseling of patients with heterozygous FBN1 pathogenic variants.

Percentage of patients with aortic event by the age of 40 to 80 years, ectopia lentis, scoliosis >20° according to their variant groups. Risk levels are illustrated by different colors (red: very high risk, orange: high risk, yellow: intermediate risk, and green: low risk). PTC premature termination codon.