Prophylactic surgery of the ascending aorta to prevent the risk of type A aortic dissection is recommended prior to pregnancy in women with Marfan syndrome (MFS) and FBN1 mutation when the aortic diameter is greater than 45mm or earlier in related syndromes.

To assess the risk of aortic dissection in pregnancy after replacement of the ascending aorta in this population.

Our study consists of a retrospective analysis of data collected at the national reference centre for Marfan and related syndromes, concerning women with MFS or related syndromes who have undergone prophylactic ascending aorta surgery before the age of 45 years and who have had at least one full-term pregnancy. The primary endpoint was the occurrence of aortic dissection in the peripartum period.

In our cohort, 23 women with MFS according to the modified Gent criteria and genetically confirmed have a history of prophylactic aortic surgery followed by pregnancy. In total, there were 49 pregnancies: 35 postoperative and 31 full term. Two postpartum aortic dissections occurred:

– one type A on day 3 in a patient with a SMAD3 mutation (dissection of the distal segment of ascending aorta),

– one type B at day 46 in a patient with an FBN1 mutation who died following descending aortic replacement surgery 1.5 years later.

No aortic dissection was observed in pre-surgical pregnancies.

Women with Marfan syndrome or related conditions who have had prophylactic ascending aortic replacement can experienced aortic dissection during pregnancy, however this risk is low (2/31; 6%).