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6.1.2.2.1. Diagnostic and Surveillance Aortic Imaging in
Marfan Syndrome
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.
If the aortic root, ascending aorta, or both are not adequately
visualized on TTE, a CT or MRI of the thoracic aorta is
recommended.
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 Aer 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.
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.
6.1.2.2.2. Medical Therapy in Marfan Syndrome
COR LOE
Recommendations
1 A 1. 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.
2a C-LD 2. 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.
