Ghent-2 Criteria (Marfan)

2010 Revised Nosology. Diagnosis requires aortic root Z-score + ectopia lentis, OR FBN1 variant, OR systemic score ≥ 7. Max systemic score = 20.

Aortic Root Z-score (TTE)

≥ 2.0 = significant

Ectopia Lentis?

FBN1 Pathogenic Variant Identified?

Systemic Score Features

Score: 0/20

Guidelines & Evidence

Verified

Last Review: 2026

Diagnosing Marfan syndrome in adults and children (threshold modifications for children < 20yr).

Distinguishing Marfan syndrome from related connective tissue disorders (MASS, MFS1, Loeys-Dietz, EDS-vascular).

Guiding decisions on genetic testing (FBN1 gene sequencing and MLPA).

Endorsed by the International Marfan Foundation, EHN, and incorporated into ESC aortic disease guidelines.

Last Comprehensive Review: 2026

Scanning Medical Journals

No new significant updates or guidelines matching this topic were found today. We will check again soon.