Table 1 Simplified revised Ghent criteria
In the absence of a family history: | Diagnosis of MFS | In the presence of a family history: | Diagnosis of MFS |
|---|---|---|---|
Aorta (Z ≥ 2 or dissection) and ectopia lentis | Yes | Ectopia lentis | Yes |
Aorta (Z ≥ 2 or dissection) and a causal FBN1 mutation | Yes | Systemic score ≥ 7 | Yes |
Aorta (Z ≥ 2 or dissection) and systemic features (≥7) | Yes | Aorta (Z ≥ 2 above 20 yr old, Z ≥ 3 below 20 yr, or dissection) | Yes |
Ectopia lentis and a causal FBN1 mutation and aortic aneurysm | Yes | Â | Yes |