Recommendations for Diagnostic and Surveillance Aortic Imaging in Marfan Syndrome

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

COR	LOE	Recommendations
Initial Diagnosis and Surveillance Imaging
1	C-EO	1. In patients with Marfan syndrome, a TTE is recommended at the time of initial diagnosis, to determine the diameters of the aortic root and ascending aorta, and 6 months thereafter, to determine the rate of aortic growth; if the aortic diameters are stable, an annual surveillance TTE is recommended.1 If the aortic root, ascending aorta, or both are not adequately visualized on TTE, a CT or MRI of the thoracic aorta is recommended.2
2a	C-EO	2. In adults with Marfan syndrome, after the initial TTE, a CT or MRI of the thoracic aorta is reasonable to confirm the aortic diameters and assess the remainder of the thoracic aorta.
Imaging After Aortic Root Replacement
1	C-LD	3. In patients with Marfan syndrome who have undergone aortic root replacement, surveillance imaging of the thoracic aorta by MRI (or CT) is recommended to evaluate for distal TAD, initially annually and then, if normal in diameter and unchanged after 2 years, every other year.3–6
2a	C-LD	4. In patients with Marfan syndrome who have undergone aortic root replacement, surveillance imaging every 3 to 5 years for potential AAA is reasonable.2,6