Recently, a young woman genetically predisposed to Ehlers-Danlos syndrome requested a semi-urgent telehealth visit. With her family history of aortic dissections and now newly pregnant, she had been told there was a 25% chance of aortic dissection. As with other serious adverse events, the chance of aortic dissection with pregnancy is often overestimated. Reporting is biased toward documenting adverse outcomes, such as dissections, and urgent successful surgical repair. A meta-analysis reported a dissection rate of nearly 8% for patients with Marfan syndrome who were pregnant, with greater rates as the proximal aortic dimensions exceed 4.0 cm.1 In patients with Ehlers-Danlos syndrome, more than 4% have been described as developing pregnancy-related events.2
McNally E. Aortic Dissection With Pregnancy—Anticipating Prepartum and Postpartum Risk. JAMA Cardiol. 2021;6(1):66–67. doi:10.1001/jamacardio.2020.4884
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