Abstract
Objective In Marfan syndrome, pregnancy is considered as high-risk due
to connective tissue insufficiency and increased risk of aortic
dissection. The objective was to demonstrate the consequences on
maternal health, including aortic events in women with Marfan syndrome.
Furthermore, to investigate outcome in undiagnosed with Marfan syndrome
at the time of pregnancy. Design Information on aortic operations,
aortic dissections, and pregnancy related outcome, from a national
cohort of women with Marfan syndrome (n=195) were compared to an
age-matched background population (n=19,500). The women with Marfan
syndrome were divided according to whether the Marfan syndrome diagnosis
was known at the time of pregnancy or not. Setting National health care
registers Methods Main outcomes measures Registered events from the
National Patient Registry in Denmark Results Significantly fewer women
with Marfan syndrome gave birth, compared to the background population.
In Marfan syndrome, complications related to cervix were increased (HR:
19.8 (95% CI: 2.2-177.5)) and the number of caesarean sections was
higher (HR: 2.09 (95% CI: 1.41-3.08)). No women with known Marfan
syndrome had a pregnancy related aortic dissection and the consequences
of pregnancy and delivery on future aortic events seemed limited. Among
undiagnosed women with Marfan syndrome there were more foetal deaths and
all delivery-related dissections came from this subgroup. Conclusion The
surveillance program for pregnant women with Marfan syndrome seems
appropriate and only women with undiagnosed Marfan syndrome experienced
a pregnancy-related aortic dissection. However, there is still an
increased risk of complications during pregnancy, but the overall
outcome data are quite reassuring.