Marfan diagnosis, aortic disease and death
Combined, 29 women with Marfan syndrome had a pregnancy registration and at least one registration with aortic disease during the study period. We did not register any women with known Marfan syndrome with an aortic dissection in close relation to delivery. Three women diagnosed with Marfan syndrome had prophylactic aortic surgery prior to a registration of delivery; none of them were registered with dissection. Twenty undiagnosed women with Marfan syndrome gave birth to 39 children.
Fifteen (out of the 29 women) had one or more aortic dissections. Their details are as follows: Nine women suffered from aortic dissection unrelated to their pregnancy registration, with the event occurring more than 15 years after the first pregnancy registration and more than 10 years after the last pregnancy registration. Three women with undiagnosed Marfan syndrome had an aortic dissection in close relation to delivery. The remaining three women each had a unique order of events, however, none dissected within 20 years after delivery.
No women with Marfan syndrome and a previous dissection had a pregnancy registration.
Six of the women with Marfan syndrome and a pregnancy registration and aortic disease died; all had a registration of an aortic operation and three had previously had a dissection. Five of these six women died more than 20 years after their first pregnancy registration. Due to the prohibited specification of single cases, we cannot report details on the sixth case. Three controls with a pregnancy registration had aortic surgery, but no dissections; none of them died.