Aortopathy in pregnancy: Unanswered questions

Aortic dissection in pregnancy has a high mortality rate for mother and fetus. Women at risk are largely those with hereditary thoracic aortic disease, that is typically undiagnosed. Existing literature has focussed on characterising the phenotype of these women, but many questions remain about the causes of dissection in pregnancy and how to best manage women antenatally and postnatally. The widespread uptake of genetic testing has allowed us to refine the genotype and we need to use this to better individualise risk. We do not know how often to screen women during pregnancy and postpartum, nor do we know whether beta-blockers affect event rates. We have very little data on the outcomes of women who have undergone aortic surgery who embark on a pregnancy, including those who have residual dissection. Passive vaginal delivery is advised for delivery, but we do not know if this is safer than elective caesarean section and there is a paucity of evidence on the effectiveness of neuraxial anaesthesia in these women. Dissection postpartum can occur days or weeks after delivery. There is little evidence on the postnatal care or blood pressure control of women who have dissected postpartum and no guidelines on best postpartum care. Lastly, we know little of the birth experience of these women and whether birth plans were successful. This paper aims to discuss some of these unanswered questions in the hope that with further research and discussion of best practice we can collectively reduce this devastating event in young women.