In the absence of family history: |
 1. Aortic Root Dilatation Z score ≥ 2 AND Ectopia Lentis = Marfan syndrome. |
 2. Aortic Root Dilatation Z score ≥ 2 AND FBN1 mutations = Marfan syndrome. |
 3. Aortic Root Dilatation Z score ≥ 2 AND Systemic Score ≥ 7pts = Marfan syndrome. |
 4. Ectopia lentis AND FBN1 with known Aortic Root Dilatation = Marfan syndrome. |
In the presence of family history: |
 1. Ectopia lentis AND Family History of Marfan syndrome (as defined above) = Marfan syndrome. |
 2. A systemic score ≥ 7 points AND Family History of Marfan syndrome (as defined above) = Marfan syndrome |
 3. Aortic Root Dilatation Z score ≥ 2 above 20 yrs. old, ≥ 3 below 20 yrs. old) + Family History of Marfan syndrome (as defined above) = Marfan syndrome. |