Aortopathies and Risk Stratification
Women with pre-existing dilatation of the aorta, or disorders that make them more susceptible to dilatation, should be monitored throughout pregnancy as they have an increased relative risk for aortic complications3. It should be noted that many aortopathies are often only diagnosed following maternal death or cardiovascular event; in the Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC) study, 42% were aware of their MFS prior to their first pregnancy5,21. The aorta is counted as dilated once the diameter exceeds the upper limit of normal; 2 standard deviations greater than the mean predicted diameter matched for age and size22.
Women with known aortopathies should have a personalised plan for care during their pregnancy, including initial pre-conception counselling on their risk23. Heritable Thoracic Aortic Disease (HTAD), Loeys-Dietz syndrome, Vascular Ehlers-Danlos (vEDS), MFS, bicuspid aortic valve (BAV) and Turner syndrome (TS) are all known risk factors for developing AAD23. An entire imaging of the aorta should be concluded prior to pregnancy by CT or MRI, as well as exercise testing, ECG and echocardiography to assess risk5. Any aortic pathology in these risk groups warrant ongoing screening; for high-risk— those likely to suffer from AD or with already dilated aortas— echocardiography every 4 weeks is necessary, but for low-risk, every 12 weeks.
Counselling on vaginal or Caesarean delivery has been discussed depending on aortic diameter; vaginal delivery is deemed safe if the ascending aorta is less than 40mm, but Caesarean delivery should be considered in all those with ascending aorta >40mm but particularly in those >45mm or a prior history of AD5.