Aortopathies
Aortic dissection is an important cause of maternal mortality [1]. The highest risk is during the last
trimester and the early post-partum period. Care in five of the seven women who died in the most
recent UK review was substandard due to inadequate investigation of chest pain. Whilst clinicians are
likely to have a higher index of suspicion in patients with known aortopathy, only three of the women
who died were known to have an aortopathy. Therefore, aortic dissection should be considered in any
woman who presents with severe chest pain.
Aortopathy may be due to inherited or connective tissue disorders such as Marfan syndrome,
LoeyseDietz syndrome (LDS), Ehlerse Danlos type IV or familial thoracic aortic aneurysm. These conditions
often have an autosomal dominant inheritance pattern, and any woman with a family history of
aortopathy or deaths during pregnancy or the post-partum period, or other features suggestive of a
connective tissue disorder should be evaluated for aortopathy and offered specialist genetic assessment
and preconception counselling. Aortic dilatation due to cystic medial necrosis also occurs in association
with congenital heart disease notably coarctation of the aorta, bicuspid aortic valve and Turner syndrome.