[Marfan's syndrome and pregnancy. A case report and review of the literature]

Abstract

Marfan's syndrome is a collagen tissue disease, identified at the end of the nineteenth century. The gene for this autosomal dominant disease lies on chromosome 15 and leads to a biochemical disorder yet to be described. We report a case of Marfan's disease in a gravid patient and discuss the complications which may occur. The main risk involves the cardiovascular manifestations of the disease with possible dissection or rupture of the aorta. Preventive surgery, with extra-corporal circulation is required when the diameter of the aorta is greater than 6 cm. Spontaneous abortions and risk of premature birth have also been reported. Delivery itself is not a major risk but should be planned in a setting allowing immediate transfer to a cardiac surgery unit. Pregnancy should be discouraged in patients with Marfan's syndrome if the aortic diameter is greater than 4 cm. When pregnancy occurs, echocardiographic examinations of the mother should be performed monthly for the first 6 months, then bimonthly. Prenatal diagnosis is of little interest, except to detect foetuses at risk at delivery.