Treatment of Unruptured Thoracic Aortic Aneurysm in Marfan Syndrome with Aortic Root or Ascending Aorta Involvement

Marfan syndrome carries a well-defined risk of aortic pathology concentrated at the aortic root. Patients develop aneurysms at the sinuses of Valsalva and face elevated risk of aortic dissection, making timely, structured management essential in this population.

Clinical scenario: Patient with Marfan syndrome presenting with aneurysm of the aortic root or ascending thoracic aorta. Patients with Marfan syndrome develop aneurysms involving the aortic root (sinuses of Valsalva) and are at risk for aortic dissection.

Management approach

Surgical management of the aortic root is the cornerstone of intervention for eligible patients in this setting. The complete protocol specifies the operative indications, the procedures considered, and the evidence supporting each — access the full regimen for details.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1161/CIR.0000000000001106

Patients with Marfan syndrome develop aneurysms involving the aortic root (sinuses of Valsalva) and are at risk for aortic dissection.

In patients with Marfan syndrome and an aortic root diameter of ≥5.0 cm, surgery to replace the aortic root and ascending aorta is recommended.

The 2 aortic root replacement procedures performed most commonly in the United States are a composite valved graft conduit and a VSRR.

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