Marfan's syndrome and pregnancy: a good maternal and fetal outcome.

Abstract

Introduction: this case report highlights the important role of a multidisciplinary team's task in the care of pregnant women with Marfan's syndrome (MFS), a systemic disorder of connective tissue that is transmitted as an autosomal dominant trait.

Case: a 42 year-old italian pregnant woman with Marfan's syndrome and degenerative heart disease (aneurysmatic dilatation of the aortic root, mitral regurgitation and prosthetic mitralic valve) was clinically assessed jointly by an obstetrician and a cardiologist, 'the obstetric team specialised in management of high risk pregnancy', every 2-3 weeks from the 21(th) week of gestation. The first ambulatory monitoring echocardiography revelead aneurysmatic dilatation of the aortic root (41 mm), good function of the previously replaced mitral valve, cardiac ejection fraction 51% and telediastolic volume 116 ml. The echocardiographies showed no changes up to 32 weeks gestation. At the 34(th) week of gestation she had a slight decrease in cardiac ejection fraction and minimal increase of left ventricular diastolic volume. Therefore she underwent elective cesarean section under general anesthesia at 35 weeks' gestation. The postpartum course was uneventfull for the patient and the baby.

Conclusion: pregnant women with heart disease benefit from an appropriate antenatal management, which may result in a favourable outcome.