Shreya Srihari
Pronouns: She/Her
Research Mentor(s): Carlos Alberto Campello Jorge
Research Mentor School/College/Department: Department of Radiology / Medicine
Program:
Authors: Shreya Srihari, Carlos Jorge, Nicholas Burris
Session: Session 1: 9:00 am – 9:50 am
Poster: 65
Abstract
Marfan syndrome (MFS) is a genetic disorder that affects the body’s connective tissue, which is essential for maintaining the structural integrity of the aorta. Individuals with MFS have an elevated risk of experiencing aortic dissection—a life-threatening condition characterized by a tear in the wall of the aorta. The risk of aortic events increases in pregnant patients with MFS due to hormonal changes and increased blood volume. This study analyzed the incidence and patterns of aortic dissection and other pregnancy-related MFS complications. We conducted a retrospective review of 38 female MFS patients who underwent two gated, multiphase CT angiography (CTA) studies with an interval of more than one year, from 2004 to 2020. The average age of the patients was 40.3 ± 14.5 years, and the mean interval between CT scans was 6.1 ± 3.6 years. Of our cohort, 15 patients had been pregnant at least once, representing 39.5% of the group. Those without a history of pregnancy were more likely to present Ectopia Lentis compared to those with prior pregnancy (39.1% vs. 20%, p = 0.02) and had a more tortuous aorta at baseline (tortuosity index: non-pregnant 1.18 vs. pregnant 1.12, p = 0.046). The aortic growth rate did not differ significantly between the groups (0.23 mm/year vs. 0.23 mm/year, p = 0.56). During the imaging follow-up, eight patients developed type B aortic dissection (TBAD), with the incidence rate being comparable between those without and with a pregnancy history (20% vs. 21.7%, p = 1.00). Despite no observed difference in aortic growth rate, the more pronounced phenotype in the non-pregnant subgroup of our MFS cohort suggests that clinical severity may influence reproductive decisions. This pattern could indicate a deliberate consideration of MFS-related risks, informed by heightened clinical awareness.
