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

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).

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.

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.