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.