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6.1.2.3.1. Imaging in Loeys-Dietz Syndrome
COR LOE
Recommendations
1 C-EO 1. In patients with Loeys-Dietz syndrome, a baseline TTE is
recommended 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, annual
surveillance TTE is recommended.
1 C-EO 2. In patients with Loeys-Dietz syndrome and a dilated or
dissected aorta and/or arterial branches at baseline, annual
surveillance imaging of the affected aorta and arteries with
MRI or CT is recommended.
1 C-LD 3. In patients with Loeys-Dietz syndrome, a baseline MRI or
CT from head to pelvis is recommended to evaluate the
entire aorta and its branches for aneurysm, dissection, and
tortuosity.
2a C-EO 4. In patients with Loeys-Dietz syndrome without dilation
of the aorta distal to the aortic root or ascending aorta and
without dilated or dissected arterial branches, surveillance
imaging from chest to pelvis with MRI (or CT) every 2 years
is reasonable, but imaging may be more frequent depending
on family history.
2a C-EO 5. In patients with Loeys-Dietz syndrome without dilation of
the cerebral arteries on initial screening, periodic imaging
surveillance for cerebral aneurysms with MRI or CT every 2
to 3 years is reasonable.
6.1.2.3.2. Medical Therapy in Loeys-Dietz Syndrome
COR LOE
Recommendation
2a C-EO 1. In patients with Loeys-Dietz syndrome, treatment with a
beta blocker or an ARB (unless contraindicated), or both, in
maximally tolerated doses, is reasonable.
