A Woman With Marfan Syndrome in Pregnancy: Managing High Vascular Risk With Multidisciplinary Care.

Background: Women with connective tissue disorders are at risk for cardiovascular complications during pregnancy, but there are no guidelines for pregnant women with aortic root diameter > 45 mm or with rapid aortic widening. We describe the issues of practical significance in the management of pregnancy and delivery in a woman with Marfan syndrome (MFS). Case: A pregnant woman with MFS presented for tertiary care at 26 weeks' gestation. Rapid aortic dilatation triggered a decision to undertake delivery preterm, with a resulting good neonatal outcome. A multidisciplinary approach aided in optimizing the monitoring and timing of delivery and subsequent aortic repair, and allowed planning for the management of a potential vascular catastrophe. Conclusion: Having optimal maternal and neonatal outcomes for pregnant women with Marfan syndrome depends on a highly responsive and coordinated team effort, including meticulous planning for a vascular catastrophe.Abstract available from the publisher.