78
Treatment
8. Pregnancy in Patients With Aortopathy
8.1. Counseling and Management of Aortic Disease in
Pregnancy and Postpartum
COR LOE
Recommendations
Prepregnancy
1 C-LD 1. In patients with genetic aortopathies attributable to syndromic
(Marfan syndrome, Loeys-Dietz syndrome, vascular Ehlers-
Danlos syndrome) and nsHTAD and who are contemplating
pregnancy, genetic counseling before pregnancy to discuss the
heritable nature of their condition is recommended.
1 C-LD 2. In patients with syndromic and nsHTAD, Turner syndrome,
BAV with aortic dilation, and other aortopathy conditions,
aortic imaging (with TTE, MRI or CT, or both as appropriate)
before pregnancy is recommended to determine aortic diameters.
1 C-LD 3. In patients with syndromic and nsHTAD, Turner syndrome,
BAV with aortic dilation, and other aortopathy conditions,
who are contemplating pregnancy, counseling about the risks
of aortic dissection related to pregnancy is recommended.
During Pregnancy
2a C-EO 4. In patients with aortic aneurysms, or at increased risk of aortic
dissection, or both, it is recommended that pregnancy be
managed by a multidisciplinary team including a maternal
fetal medicine specialist and cardiologist, and, if logistically
feasible, that delivery be planned in a hospital where the
capability for emergency aortic repair is available.
1 C-LD 5. In patients with aortopathies who are pregnant, guideline-
directed treatment of hypertension is recommended.
1 C-EO 6. In patients with syndromic and nsHTAD, beta-blocker
therapy during pregnancy and postpartum is recommended,
unless contraindicated.
1 C-LD 7. In pregnant patients with an aortopathic condition or a dilated
aortic root or ascending aorta, surveillance TTE to monitor
aortic diameters and aortic valve function is recommended
each trimester and again several weeks postpartum, although
imaging may be more frequent depending on aortic diameter,
aortic growth rate, and underlying condition.
1 C-LD 8. In pregnant patients with aortic disease who require
surveillance imaging of the aortic arch, descending, abdominal
aorta, or all 3, MRI without gadolinium is recommended over
CT to avoid radiation exposure to the fetus.
