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- 저자 : ( Kylie Hae-hin Chang ) (검색결과 1 건)
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Marfan syndrome and pregnancy; maternal and neonatal outcomes
( Hyein Jang ),( Mina Kim ),( Kylie Hae-hin Chang ),( Ji-hee Sung ),( Suk-joo Choi ),( Soo-young Oh ),( Cheong-rae Roh ),( Jong-hwa Kim ) 대한산부인과학회 2016 대한산부인과학회 학술대회 Vol.102 No.-
Objective: To report characteristics and pregnancy outcomes of recent series of Marfan syndrome (MFS) in tertiary care center over a 12-year period. Methods: A retrospective case review of 13 pregnant women with MFS who admitted to our institution (MFS clinic). Maternal characteristics including age at first diagnosis of MFS and gestational age at delivery and cardiac variables relating MFS including pre-pregnancy and pre-delivery diameter of sinus of valsalva, event of aortic dissection were collected. We also examined the fibrilin-1 (FBN 1) gene mutation of mother and neonate. Results: During 12-year period, 13 pregnant women with MFS admitted. The mean age at pregnancy and the diagnosis of MFS was 31.5 and 25.9 year old, respectively. Two women had termination of pregnancy because of high risk of aortic dissection (aortic diameter; 44 mm and 45.5mm). The median aortic diameter of ongoing pregnancy was 32.7 mm [range: 26-40mm]. There were two women with missed abortion. All ongoing pregnancies were delivered by cesarean section either elective or emergent mode. Four patients delivered at term and another four patients delivered in preterm. Except one case, twelve women were diagnosed as MFS before pregnancy and had pre-conceptional multidisciplinary counseling including beta-blocking agents use and inheritance. In one case who was not diagnosed before pregnancy, acute type A aortic dissection occurred at 29+1 week and she had an emergency cesarean section with Bentall operation. There were no maternal deaths. Among 13 women, 11 underwent FBN 1 mutation test and 8 of out 11 showed positive mutation of FBN 1 gene. There were four babies who had mutation in FBN 1 gene and one baby expired because of severe congenital MFS. Conclusions: Although pregnancy in women with MFS increases the risk of significant cardiac, obstetric and neonatal complications, proper pre-conceptional counselling with multidisciplinary approach can decrease maternal morbidity and mortality.
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