Pregnancy and Childbirth in Patients With Syndrome-Marfan
DOI:
https://doi.org/10.17140/GOROJ-4-137Keywords:
Marfan syndrome, Aortic root enlargement, Ectopia lentis, Fibrillin-1, Beta blockers, Dural ectasia, Protrusio acetabuliAbstract
Marfan syndrome (MFS) is an autosomal dominant condition with a reported incidence rate of 1 in 3000 to 5000 individuals. The majority of cases of MFS are caused by a mutation in the fibrillin-1 gene (FBN1). Transforming growth factor β (TGF-β) plays an important role in Marfan syndrome. The identification of the FBN-1 mutation will help identify potentially affected family members and promote prenatal diagnostic testing. β blockers decrease myocardial contractility and pulse pressure and may also improve the elastic properties of the aorta. Angiotensin II-receptor blockers attenuate the clinical manifestations of MFS.
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