Type IV Vascular Ehlers-Danlos
vEDS is an autosomal dominant condition from mutations of
COL3A131. 86.7% of women were diagnosed with vEDS
after completion of their pregnancy31. For patients
with vEDS, pregnancy is contraindicated due to the high risks of
dissection and uterine rupture5. However, this
recommendation was based off a study that found there was no survival
difference between nulliparous vEDS women and parous vEDS. Murrayet al . looked at maternal morbidity in their study of 565
pregnancies. Their mortality rate of pregnancy-related deaths was 5.3%
(30). Arterial dissection or rupture occurred in 9.2%, including 3
fatal aortic ruptures, 2 during labour at term, and 1 postpartum.
Comparing Kaplan-Meier survival estimates of nulliparous versus parous
vEDS and comparing groups of women not diagnosed due to complications
and adjusting for mutation type, the mean age of death was approximately
the same at 53 years31. This led Murray et al .
to conclude that the decision of pregnancy in vEDS should be based on
expert discussion and planning with the patient involved, and not
contraindicated based on genetic diagnosis. The use of celiprolol, a
B1-adrenoceptor antagonist, has also been noted to improve
cardiovascular adverse events and could be utilised during pregnancy,
subject to fetal monitoring22.