Recommendations for 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.1–4 |
| 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.1–3,5–13 |
| 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.2–5,10,12,14 |
| 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.6,15,17 |
| 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.7–9,17,18 |
| 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.19,20 |