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.