Table 2. Components of the revised Ghent nosology in patients with MFS.
Events/available data | % | |
---|---|---|
Ao (Z score ≥2) | 303/342 | 88.6 |
Ectopia lentis | 163/296 | 55.1 |
FBN1 gene mutation | 141/157* | 89.8 |
Systemic score ≥7 | 217/294 | 73.8 |
Family history of MFS | 97/218 | 44.5 |
*Number of available data among the probands. †Number of probands. MFS: Marfan syndrome, Ao: aortic diameter at the sinus of Valsalva above indicated Z-score or aortic root dissection, FBN1: Fibrillin-1