Maternal complication of pregnancy in Marfan syndrome.

OBJECTIVES

Evaluate the risk of aortic complications (aortic dissection or prophylactic aortic surgical replacement) associated with pregnancy in Marfan syndrome in the absence of specific care.

BACKGROUND

Data are scarce on aortic complications during pregnancy in Marfan syndrome.

METHODS

Retrospective study on a large population (415 patients) followed up in our multidisciplinary out-patient clinic devoted to Marfan syndrome. Women over 18 years of age were divided into 2 groups: 85 had been pregnant (MFSP+) giving birth to 136 children through 160 pregnancies; 68 had not been pregnant (MFSP-). The occurrence of aortic complication was compared between the 2 groups using a piecewise discrete-time model.

RESULTS

In MFSP+, 7 aortic complications occurred during 160 pregnancies (4.4%) and 17 aortic complications occurred during 1870 years of follow-up out of pregnancy. In MFSP-, 14 aortic complications occurred over 940 years of follow-up. Pregnancy was associated with a 5 fold increase in risk of aortic complication, which did not translate into increased risk during life, suggesting that pregnancy may act as a revealer in women prone to aortic complication.

CONCLUSIONS

Pregnancy is associated with a transient increased risk of aortic complication in the absence of specific care.