Inherited aortopathies and risk of aortic dissection and aortic syndrome in pregnancy

Acute aortic dissection related to pregnancy is rare, however represented the third most common cause of pregnancy-related cardiovascular death in the 2017 UK-MBBRACE report. The majority of women with pregnancy-related aortic dissection have an underlying inherited syndromic aortopathy, though this is often not recognised until an event. Data regarding the immediate and long-term effects of pregnancy on aortic outcome are not uniform due to publication and ascertainment biases, small sample sizes, inclusion of women whose dissection was the first presentation of the underlying disease, and incompleteness of collected data. Management recommendations are based on relatively low levels of evidence, and there is some variation between society guideline recommendations. This article seeks to review the available evidence regarding pregnancy-related aortic dissection in women inherited syndromic aortopathies, highlighting the importance of pre-conception counselling, genetic testing and a multi-disciplinary team approach to management.