A prospective longitudinal evaluation of pregnancy in the Marfan syndrome

Abstract

Objective: We undertook a prospective evaluation of the outcomes of pregnancy, both maternal and fetal, and the long-term impact of pregnancy on Marfan syndrome in a series of consecutive, unselected patients.

Study design: Forty-five pregnancies in 21 Marfan syndrome patients were prospectively observed in one institution between 1983 and 1992. During pregnancy, patients were monitored with serial echocardiograms and close attention to symptoms. Maternal and fetal outcomes were monitored with serial echocardiographic data were analyzed by least-squares regression. Eighteen of the patients were followed up for 15 months to 13 years after the completion of their last pregnancy for investigation of the long-term impact of pregnancy on the cardiovascular manifestations of Marfan syndrome.

Results: Aortic dissection occurred in two patients, both with increased risk for dissection established before pregnancy. The incidence of obstetric complications otherwise did not exceed that in the general population. Echocardiographic data demonstrated little to no change in aortic root diameter throughout pregnancy in most patients. Long-term follow-up showed no apparent worsening of cardiovascular status attributable to pregnancy in comparison with a group of 18 women with Marfan syndrome who were of similar age, had a similar degree of disease severity, and underwent no pregnancies.

Conclusions: Patients with Marfan syndrome in whom cardiovascular involvement is minor and aortic root diameter is < 40 mm usually tolerate pregnancy well, with favorable maternal and fetal outcomes, and without subsequent evidence of aggravated aortic root dilatation over time.