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Background  The risk of aortic complications associated with pregnancy in women with Marfan syndrome ( MFS ) is not fully understood.    Methods and Results   MFS  women participating in the large National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (Gen TAC ) were evaluated. Among 184 women with  MFS  in whom pregnancy information was available, 94 (51%) had a total of 227 pregnancies. Among the women with pregnancies, 10 (10.6%) experienced a pregnancy‐related aortic complication (4 type A and 3 type B dissections, 1 coronary artery dissection, and 2 with significant [≥3 mm] aortic growth). Five of 7 aortic dissections, including all 3 type B, and the coronary dissection (75% of all dissections) occurred in the postpartum period. Only 5 of 8 women with pregnancy‐associated dissection were aware of their  MFS  diagnosis. The rate of aortic dissection was higher during the pregnancy and postpartum period (5.4 per 100 person‐years vs 0.6 per 100 person‐years of nonpregnancy; rate ratio, 8.4 [95% CI=3.9, 18.4];  P <0.0001).    Conclusions  Pregnancy in  MFS  is associated with an increased risk of aortic dissection, both types A and B, particularly in the immediate postpartum period. Lack of knowledge of underlying  MFS  diagnosis before aortic dissection is a major contributing factor. These findings underscore the need for early diagnosis, prepregnancy risk counseling, and multidisciplinary peripartum management.

journal of the american heart association

Aortic Complications Associated With Pregnancy in Marfan Syndrome: The  NHLBI  National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (Gen TAC )