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Background: Marfan syndrome is a heritable connective tissue disorder with significant risk of cardiovascular compromise. The physiological changes of pregnancy greatly increases that risk resulting in significant threat of maternal and fetal morbidity and mortality. Case: A 21-year-old G1P0 female with Marfan syndrome presented at 16 weeks gestation with a 7.3cm ascending aortic aneurysm and a desire to maintain her pregnancy. Immediate surgical repair was performed upon presentation with reassuring maternal and fetal status until cesarean section delivery at 39 weeks gestation. The patient’s postpartum course was complicated by an expanding descending aortic aneurysm which, upon advancement to a type B aortic dissection, required additional surgical management. Conclusion: The risk of cardiovascular sequelae associated with Marfan syndrome in pregnancy is significant though it can be lessened with preconception counseling and potential surgical intervention. In the event of cardiovascular compromise during pregnancy, endovascular or surgical repair versus medical management are treatment options dependent on patient presentation, acuity and available resources.

An Uncommon Case of Antepartum and Postpartum Surgical Aorta Repair in a Patient with Marfan Syndrome