Marfan Surgery

Guest: Alberto Pochettino, M.D.

Hosts: Paul A. Friedman, M.D.

Marfan syndrome is a genetic abnormality where the fibrillin protein is abnormal. That affects all collagen structures within a given individual. Phenotypic features involve ocular abnormalities such as lens dislocation and a higher risk of retinal detachment, as well as diffuse musculoskeletal abnormalities such as scoliosis, joints abnormalities, and the diffuse hyper extension of all joints. By far the most life-threatening feature in Marfan syndrome is aortic abnormality.

Topics Discussed:

Which patients with Marfan syndrome should be evaluated for aortic disease?
Do all Marfan patients develop aortic aneurysm?
What other cardiovascular abnormalities are noted in Marfan syndrome?
What preventive operations are available, and what are the expected outcomes from such operations?
If a Marfan patient develops a type B dissection what is the appropriate management?

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Podcast episode transcript found here.

Marfan Syndrome