At what aortic diameter is elective repair recommended in Marfan?

Elective aortic root/ascending replacement at ≥45 mm (or Z-score ≥3 in children/adolescents); earlier at ≥40 mm if rapid growth (>3 mm/year), family history of dissection, or severe AR.

What BSA-indexed aortic root Z-score is normal?

Z-score <2 is normal; Z-score 2-3 is borderline/monitoring; Z-score ≥3 warrants increased surveillance; surgical planning typically at Z-score ≥4-5 depending on growth rate and clinical features.

How does losartan reduce aortic root growth in Marfan?

Losartan (ARB) reduces TGF-β signaling, the key pathogenic pathway in Marfan aortopathy; clinical trials show modest benefit; combined beta-blocker + ARB is recommended in most guidelines.

What activities are restricted in Marfan syndrome?

Contact sports, competitive sports with high static/dynamic component, weightlifting, and scuba diving are contraindicated; swimming, walking, and low-intensity aerobics are encouraged.

What is the 2010 revised Ghent nosology for Marfan diagnosis?

2010 Ghent: FBN1 mutation + aortic root Z≥2 = Marfan confirmed; aortic root Z≥2 + systemic score ≥7 = Marfan; ectopia lentis + FBN1 mutation causing aortic disease = Marfan.

Marfan Syndrome Aortic Root Dissection Risk Calculator

Calculates aortic dissection risk in Marfan syndrome using aortic root Z-score (age/BSA-indexed diameter), family history of dissection, and rate of aortic growth to determine surgical threshold.

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Frequently Asked Questions

At what aortic diameter is elective repair recommended in Marfan?

What BSA-indexed aortic root Z-score is normal?

How does losartan reduce aortic root growth in Marfan?

What activities are restricted in Marfan syndrome?

What is the 2010 revised Ghent nosology for Marfan diagnosis?