Marfan Surgery

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.