Abstract 11940: Aortic Complications Associated With Pregnancy in Marfan Syndrome: The NHLBI GenTAC Registry

Abstract

Background: Definition of pregnancy risk in women with Marfan syndrome (MFS) has been confounded by ascertainment bias, lack of imaging data, small sample size, and inclusion of women without a known MFS diagnosis before a pregnancy complication. To lessen these limitations, we analyzed MFS women participating in the large GenTAC Registry. Results: Among 184 women in whom pregnancy information was available, 94 women (51%) had a total of 227 pregnancies (average 2.5, range 1-6). Compared to MFS women who never became pregnant, those with pregnancies were older at time of MFS diagnosis (27 vs. 13 years, p<0.0001) and at time of GenTAC enrollment (47 vs. 37 years, p<0.0001). When comparing ever-pregnant to never-pregnant women with MFS at the time of registry entry, higher historical rates of aortic dissection and prophylactic proximal aortic surgery were observed (24/94 [25.5%] vs. 13/90 [14.4%], p=0.068 and 15/94 [16.0%] vs. 4/90 [4.4%], p=0.014; respectively). Among the 94 MFS women with pregnancies, 10 (10.6%) experienced a pregnancy-related aortic complication (4 Type A and 3 Type B dissections, one coronary artery dissection, and 2 with significant [≥3 mm] aortic growth, 1 of whom required post-partum prophylactic surgery). Four of the aortic dissections, including all 3 Type B, and the coronary dissection occurred in the post-partum period. Only 3 of 7 women (43%) with an aortic dissection related to pregnancy were aware of their MFS diagnosis before pregnancy. Women with and without pregnancy-associated aortic complications did not differ in phenotypic features. The rate of aortic dissection was higher during pregnancy + post-partum period (3.5 per 100 person-years vs. 0.8 per 100 person-years, odds ratio 4.6 [95% confidence interval 2.1-10.1], p=0.0002). Conclusions: Pregnancy in MFS is associated with an increased risk of aortic dissection, both Types A and B, particularly in the immediate post-partum period, and more frequent prophylactic surgery. Lack of knowledge of underlying MFS diagnosis before aortic dissection is a major contributing factor. These findings underscore the need for early diagnosis, pre-pregnancy risk counselling, and multi-disciplinary peri-partum management.