Recommendations for Marfan Syndrome Interventions: Replacement of the Aortic Root in Patients With Marfan Syndrome

Referenced studies that support the recommendations are summarized in the Online Data Supplement.

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.1–4
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.1,3,4
2a	C-LD	3. In patients with Marfan syndrome and a maximal cross-sectional aortic root area (cm2) to patient height (m) ratio of ≥10, surgery to replace the aortic root and ascending aorta is reasonable, when performed by experienced surgeons in a Multidisciplinary Aortic Team.5
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.2–4