The Marfan syndrome and pregnancy: a retrospective study in a Dutch population

Objective: To assess the course and outcome of pregnancies in women with the Marfan syndrome with the aim of developing guidelines for counseling. Study design: A retrospective study based on data collected from members of the Dutch Association of Marfan patients. Pregnancies and neonatal outcomes of affected mothers were compared with those of non-affected mothers who delivered a Marfan infant. Results: In a group of 44 affected women 78 pregnancies beyond 24 weeks of gestation were evaluated, compared with 51 in non-affected women. Obstetric course and neonatal outcome of pregnancy were similar in both groups. Aortic dissection was observed in five affected women, three of which were known to have an aorta diameter of 40 mm or more; two neurovascular events were recorded; all mothers survived. Conclusions: A preconceptional aortic diameter of 40 mm or more, progression of dilatation and decreased cardiac function are risk factors in pregnancy for women with the Marfan syndrome. A multidisciplinary approach is recommended for the care of these patients and their infants.