Connective tissue disorders such as Marfan syndrome, Loeys-Dietz syndrome, and Ehlers-Danlos syndrome weaken the structural integrity of the aortic wall, raising the risk of aortic dissection during pregnancy and the postpartum period. This risk creates a critical planning requirement for cardiovascular surgery and intensive care teams managing pregnancy in these patients.
A retrospective cohort study using Japan's nationwide administrative claims database (Diagnosis Procedure Combination) analyzed 226 deliveries by 175 women with Marfan-related disorders between 2010 and 2023. Cesarean section accounted for 69.0% of deliveries. During pregnancy, 1.8% of cases (4/226) experienced aortic dissection requiring aortic surgery; during postpartum hospitalization, 2.7% of cases (6/226) experienced aortic dissection. Within one year after delivery, 2.7% of cases (6/226) required re-hospitalization for aortic dissection.
The study also examined the use of cabergoline, administered within two days postpartum for elective avoidance of breastfeeding in 6.6% of cases; the incidence of postpartum aortic dissection did not differ significantly between cabergoline users and non-users.
The findings indicate that women with Marfan-related connective tissue disorders may remain at risk of aortic dissection for up to one year postpartum, suggesting that postpartum surveillance should not be limited to the early puerperium alone.
Source: Aortic dissection during the perinatal period in women with Marfan-related disorders: a retrospective cohort study using the Japanese Diagnosis Procedure Combination database, The Journal of Maternal-Fetal & Neonatal Medicine, 2026.