32
Treatment
6.1.2.2.3. Marfan Syndrome Interventions: Replacement of
the Aortic Root in Patients With Marfan Syndrome
COR LOE
Recommendations
1 B-NR 1. 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.
2a B-NR 2. In patients with Marfan syndrome, an aortic root diameter
of ≥4.5 cm, and features associated with an increased risk of
aortic dissection (see Table 10), surgery to replace the aortic
root and ascending aorta is reasonable, when performed by
experienced surgeons in a Multidisciplinary Aortic Team.
2a C-LD 3. In patients with Marfan syndrome and a maximal cross-
sectional aortic root area (cm
2
) to patient height (m) ratio
of greater than or equal to ≥10, surgery to replace the aortic
root and ascending aorta is reasonable, when performed by
experienced surgeons in a Multidisciplinary Aortic Team.
2b C-LD 4. In patients with Marfan syndrome and an aortic diameter
approaching surgical threshold, who are candidates for valve-
sparing root replacement (VSRR) and have a very low surgical
risk, surgery to replace the aortic root and ascending aorta
may be reasonable when performed by experienced surgeons
in a Multidisciplinary Aortic Team.
6.1.2.2.4. Marfan Syndrome Interventions: Replacement
of Primary (Nondissected) Aneurysms of the Aortic Arch,
Descending, and Abdominal Aorta in Patients With Marfan
Syndrome
COR LOE
Recommendation
2a C-EO 1. In patients with Marfan syndrome and a nondissected
aneurysm of the aortic arch, descending thoracic aorta, or
abdominal aorta of ≥5.0 cm, surgical intervention to replace
the aneurysmal segment is reasonable.
Table 10. Features Associated With Increased Risk of Aortic
Complications in Marfan Syndrome
Family history of aortic dissection
Rapid aortic growth (≥0.3 cm/y)
Diffuse aortic root and ascending aortic dilation
Marked vertebral arterial tortuosity
