OUTCOME OF PREGNANCY IN WOMEN WITH MARFANS-SYNDROME

Objective To improve life expectancy and prevent premature mortality i n women with Marfan's syndrome. Methods During the development of a re gional genetic register for Marfan's Syndrome the outcome of 91 pregna ncies in 36 women with this condition was established retrospectively and the cardiovascular and obstetric complications documented. Results No patient had a significant cardiovascular abnormality limiting func tion before her pregnancy. Of 36 women, four had an aortic dissection relating to pregnancy and two others required aortic surgery following delivery. Thirty women had uncomplicated gestational histories. The i ncidence of obstetric complications did not exceed expectation. Conclu sions Women with Marfan's syndrome are at significant risk of aortic d issection in pregnancy even in the absence of preconceptional cardiova scular abnormality. Aortic root dilatation may be a predictor of risk but dissection may occur without significant dilatation. Guidelines fo r obstetric care are suggested and preconceptional assessment recommen ded.