TABLE 1

Revised Ghent Diagnostic Criteria for Marfan Syndrome

Diagnosis of definitive Marfan syndrome (any of the following)
 • Aortic root ≥2 z score and ectopia lentis
 • Aortic root ≥2 z score and FBN1 mutation
 • Aortic root ≥2 z score and systemic score ≥7
 • Ectopia lentis and FBN1 mutation known to be associated with Marfan syndrome
 • Positive family history of Marfan syndrome and ectopia lentis
 • Positive family history of Marfan syndrome and systemic score ≥7
 • Positive family history of Marfan syndrome and aortic root ≥3 z score in those <20 y of age or ≥2 z score in those >20 y of age
Diagnosis of potential Marfan syndrome
 • FBN1 mutation with aortic root with a z score <3 in those <20 y of age