Interpretation
It is well known that Marfan syndrome in general is associated with grossly increased risk of aortic disease7. Prior studies on Marfan syndrome and pregnancy have primarily been focusing on aortic events in pregnant women already diagnosed with Marfan syndrome and cared for in tertiary centres12.
In accordance with previous research, we found an increased risk of aortic events in Marfan syndrome women, however all dissections during pregnancy or delivery were in undiagnosed Marfan women.
In the women diagnosed with Marfan syndrome, we identified a significantly decreased number of registrations of non-complicated deliveries as well as significantly more extra-uterine pregnancies and caesarean sections, indicating that a pregnancy in a woman with Marfan syndrome is indeed more complicated than a normal pregnancy. The findings related to caesarean section is expected due to the definition of pregnancy in Marfan syndrome being a high risk pregnancy and the recommendation of caesarean section at aortic diameter of ≥40 mm.13 We consider the findings of a significantly reduced number of non-complicated deliveries a logic consequence hereof, as pregnancies and deliveries in women with Marfan syndrome per se are considered high risk.
Surprisingly, the findings related to the connective tissue component of the birth canal, here interpreted as findings related to uterine, cervical, and vaginal complications, were not as frequent as expected. Although complication related to the cervix was significantly increased, there was no significantly increased rate of complications related to the uterine cavity or the vagina. However, in women without a pre-pregnancy diagnosis of Marfan syndrome there was an increased risk of vaginal complications, and among women with a pre-pregnancy diagnosis there were an increased risk of cervical complications.
The significantly increased number of extra uterine pregnancies is an unexpected finding, not previously reported. Here, we identified an increased risk in Marfan syndrome women diagnosed at the time of registration, however the risk in women with Marfan syndrome not yet diagnosed was not increased. Combined, the association was not significant.