Pregnancy with Marfan syndrome (MFS) presents challenges to the clinician because of the increased incidence of maternal cardiovascular complications, including acute aortic dissection (ADD) and the involvement of the fetus owing to the urgency of the need for surgery and the low likelihood of survival with premature delivery. Patient 1 was a 36-year-old pregnant Japanese woman with MFS who was referred at 16 gestational weeks with aortic root diameter 35.1 mm. Stanford type B AAD occurred on delivery without evident blood pressure changes. Patient 2 was a 31-year-old pregnant Japanese woman with MFS with aortic root diameter 28.3 mm at 20 gestational weeks after David procedure. Ultimately fatal Stanford type B AAD occurred on post-partum day 15. Both patients were treated with beta-blockers when AAD occurred. Although these two patients were initially considered to have relatively low risk according to previous reports and guidelines, AAD occurred. It is very important to provide information about these high pregnancy risks to women with MFS before conception, and to follow MFS pregnancies with great caution.
Keywords: Marfan syndrome; aortic dissection; intrapartum; post-partum; pregnancy.
© 2017 Japan Society of Obstetrics and Gynecology.