Aortopathies and Vascular Complications

Pregnancy for women with aortopathies and connective tissue disorders has historically been considered high to prohibitive risk. However, there is increasing evidence pregnancy is better tolerated when closely managed by a multidisciplinary team. Improving pregnancy outcomes starts prior to pregnancy by ensuring women are medically optimized and considering prophylactic aortic replacement when aortic diameter is >40–45 mm in Marfan syndrome, >2.5 cm/m2 in Turner’s syndrome, or >50 mm with a bicuspid aortic valve. During pregnancy, hypertension is the most common complication, with beta-blockers generally the preferred medical therapy for women with aortopathies. If aortic size is <40 mm, a vaginal birth with epidural anesthesia is recommended and can still be considered for women with aortic size 40–45 mm, except for women with prior aortic dissections or vascular Ehlers-Danlos syndrome. Otherwise, cesarean delivery is the preferred method of delivery. Aortic dissection is the most serious complication for this population. However, in contemporary series most aortic dissections occur in women without a previously diagnosed aortopathy. The one exception is post-partum type B aortic dissections in Marfan syndrome and Loeys-Dietz syndrome which can occur in patients without significant aortic dilation and on beta-blocker therapy. When aortic dissections do occur during pregnancy, overall maternal survival is extremely high with slightly reduced rates of fetal survival. If the fetus can be delivered, cesarean delivery followed by standard management for either Stanford type A or type B aortic dissections is recommended. Otherwise, surgery with fetus in-utero for type A dissections or complicated type B aortic dissections is recommended.