Treatment of Unruptured Thoracic Aortic Aneurysm in Marfan Syndrome with Aortic Root Involvement
Patients with Marfan syndrome are at increased risk for aneurysms arising at the aortic root and ascending thoracic aorta, with progressive dilation carrying risk of dissection. Structured management in this population is directed at limiting that progression.
This protocol addresses unruptured thoracic aortic aneurysm specifically in patients with Marfan syndrome where the aneurysm involves the aortic root (sinuses of Valsalva) or ascending thoracic aorta — a population at recognised risk for aortic dissection.
Treatment Approach
Beta-blocker therapy plays a central role in reducing the rate of aortic dilation in this population — the complete protocol specifies agent selection, the circumstances under which alternative or additional pharmacological approaches apply, and how contraindications are handled.
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, treatment with either a beta blocker or an ARB, in maximally tolerated doses (unless contraindicated), is recommended to reduce the rate of aortic dilation.
In patients with Marfan syndrome, the use of both a beta blocker and an ARB, in maximally tolerated doses (unless contraindicated), is reasonable to reduce the rate of aortic dilation.
View source ↗