title: [Marfan syndrome in pregnancy: presentation of four cases and discussion].
abstract: The coincidence of Marfan syndrome and pregnancy means a high risk for mother and child, as it is associated with cardiovascular and obstetric complications. We report our experience of four pregnancies with the Marfan syndrome. The course of pregnancy, the peripartum management and both the maternal and neonatal outcomes of four pregnant women with the Marfan syndrome, who were treated in our department between 1995 and 2005, were retrospectively analysed. The pregnancies of two women were complicated by premature rupture of membranes (36 (th) gestational week) and premature uterine contractions with cervical incompetence (30 (th) gestational week), respectively. One patient developed class 3 (NYHA) heart failure in the 3 (rd) trimenon. Two out of four women had mild cardiovascular disease and could deliver vaginally. In the other two cases a primary Caesarean section was performed at the 36 (th) week of gestation because of severe cardiovascular morbidity. No patient had a progressive aortic dilatation, dissection or rupture. The neonatal outcome was uneventful in all cases. Three newborns underwent a genetic evaluation for the Marfan syndrome, in two of them mutations in the fibrillin 1 gene were detected. Women with the Marfan syndrome should be counselled pre-conception and observed by an interdisciplinary team during pregnancy. If the aortic root diameter is < 40 mm, without progression in pregnancy, and in the absence of severe valve insufficiency, then pregnancy is in most cases well tolerated and vaginal delivery can be performed.
authors.affiliation: Medizinische Hochschule Hannover, Zentrum Frauenheilkunde, Abteilung I: Frauenheilkunde und Geburtshilfe. tzialidou.irini@mh-hannover.de
authors.forename: I
authors.initials: I
authors.lastname: Tzialidou
authors.forename: K
authors.initials: K
authors.lastname: Oehler
authors.forename: A
authors.initials: A
authors.lastname: Scharf
authors.forename: I
authors.initials: I
authors.lastname: Staboulidou
authors.forename: M
authors.initials: M
authors.lastname: Westhoff-Bleck
authors.forename: P
authors.initials: P
authors.lastname: Hillemanns
authors.forename: H H
authors.initials: HH
authors.lastname: Günter
mesh_terms: D000328:Adult; D000784:Aortic Dissection / Q000175:diagnosis / Q000235:genetics; D017544:Aortic Aneurysm, Abdominal / Q000175:diagnosis / Q000235:genetics; D002585:Cesarean Section; D004252:DNA Mutational Analysis; D005260:Female; D005322:Premature Rupture of Fetal Membranes / Q000175:diagnosis; D000071838:Fibrillin-1; D000071837:Fibrillins; D005817:Genetic Counseling; D005820:Genetic Testing; D006333:Heart Failure / Q000175:diagnosis / Q000235:genetics; D006801:Humans; D007231:Infant, Newborn; D008382:Marfan Syndrome / Q000175:diagnosis* / Q000235:genetics; D008840:Microfilament Proteins / Q000235:genetics; D007744:Obstetric Labor Complications / Q000175:diagnosis* / Q000235:genetics; D007752:Obstetric Labor, Premature / Q000175:diagnosis / Q000235:genetics; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis* / Q000235:genetics; D011249:Pregnancy Complications, Cardiovascular / Q000175:diagnosis / Q000235:genetics; D011263:Pregnancy Trimester, Third; D018566:Pregnancy, High-Risk; D012189:Retrospective Studies; D002581:Uterine Cervical Incompetence / Q000175:diagnosis / Q000235:genetics; D014620:Vacuum Extraction, Obstetrical; D054392:Adipokines
chemical_list: D000071838:Fibrillin-1; D000071837:Fibrillins; D008840:Microfilament Proteins; C000605722:FBN1 protein, human; D054392:Adipokines