title: Three pregnancies in a Marfan syndrome patient after a mitral and tricuspid valve surgery.
abstract: 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.
authors.affiliation: Department of Gynecology, Obstetrics and Gynecological Oncology, Bytom, Medical University of Silesia, Katowice, Poland.
authors.forename: Jacek
authors.initials: J
authors.lastname: Zamłyński
authors.forename: Anita
authors.initials: A
authors.lastname: Olejek
authors.forename: Alicja
authors.initials: A
authors.lastname: Gajewska-Kucharek
authors.forename: Ewa
authors.initials: E
authors.lastname: Horzelska
authors.forename: Michael P
authors.initials: MP
authors.lastname: Carson
authors.forename: Monika
authors.initials: M
authors.lastname: Rykaczewska-Czerwińska
authors.forename: Jerzy
authors.initials: J
authors.lastname: Chudek
authors.forename: Piotr
authors.initials: P
authors.lastname: Bodzek
mesh_terms: D000328:Adult; D017545:Aortic Aneurysm, Thoracic / Q000601:surgery*; D001021:Aortic Valve / Q000601:surgery*; D002585:Cesarean Section; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008382:Marfan Syndrome / Q000601:surgery*; D008943:Mitral Valve / Q000601:surgery; D008944:Mitral Valve Insufficiency / Q000601:surgery*; D010298:Parity; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000517:prevention & control*; D011256:Pregnancy Outcome