Three pregnancies in a Marfan syndrome patient after a mitral and tricuspid valve surgery

Marfan syndrome is an autosomal dominant disorder of connective tissue with up to 25% of cases related to a spontaneous mutation. It has been associated with perinatal loss, preterm labor and, potentially a rupture of the maternal aortic arch. We present a case of a woman diagnosed with Marfan syndrome after a miscarriage of her first pregnancy. At the time of diagnosis she had mild aortic bulb dilation and insufficiency of the mitral and tricuspid valves. She underwent cardiosurgical correction, after which she had two uneventful pregnancies. This case suggests that preconceptional correction of valve defects in women with Marfan syndrome may decrease the risk of cardiac decompensation during future pregnancies. Additionally close clinical follow up and the appropriate use of beta-adrenergic blockade may decrease the risk of aortic rupture, a significant risk factor for mortality in pregnant women.