OBJECTIVE:The aim was to investigate birth characteristics, obstetric and neonatal outcomes of the first childbirth in women with Marfan syndrome by use of Swedish national registers since pregnancy-related outcomes in women with Marfan syndrome have only been sparsely investigated. STUDY DESIGN:In this national population-based cohort study and matched case-control study of Swedish women born 1973-1993, women with Marfan syndrome (n=273) were compared to women without the condition (n=1 017 265). The study population was followed until 2013. A total of 364 553 mother-firstborn-offspring pairs were analyzed. Sixty-one women with Marfan syndrome became mothers. Women with Marfan syndrome were also compared to 543 healthy controls. RESULTS:Women with Marfan syndrome were more often born preterm (p<0.001), small-for-gestational age (p<0.001), and delivered by cesarean section (p=0.001). Women with Marfan syndrome had no increased risk of giving birth by cesarean section (p=0.079). No increased neonatal risks in their children were found. Women with Marfan syndrome were less likely to give birth than those without (p<0.001). There were no maternal deaths. CONCLUSIONS:Women with Marfan syndrome were more likely to be born preterm, SGA and by cesarean section. These increased risks of preterm birth and SGA babies were not found in connection with their own first childbirth. Pregnancies with known fetal Marfan syndrome have to be carefully monitored. The results are important for obstetricians giving preconception counseling and treating women with Marfan syndrome. Further studies are needed to evaluate risks during pregnancy and long-term effects of pregnancy on the cardiovascular status of women with Marfan syndrome.

译文

目的:由于仅对马凡氏综合征妇女的妊娠相关结局进行了稀疏调查,因此目的是通过瑞典国家登记簿调查马凡氏综合征妇女的首次生育的​​出生特征,产科和新生儿结局。
研究设计:在这项基于全国人群的队列研究和匹配的病例对照研究中,对1973年至1993年出生的瑞典妇女进行了比较,将患有马凡氏综合症(n = 273)的妇女与未患此病的妇女(n = 1 017 265)进行了比较。研究人群一直追踪到2013年。共分析了364 553对母亲-第一胎-后代。六十一名患有马凡氏综合症的妇女成为母亲。还比较了患有马凡氏综合症的女性和543名健康对照者。
结果:马凡氏综合征的妇女更早产(p <0.001),小胎龄(p <0.001),并通过剖宫产分娩(p = 0.001)。患有马凡氏综合症的妇女通过剖宫产术分娩的风险没有增加(p = 0.079)。没有发现孩子的新生儿患病风险增加。患有马凡氏综合症的妇女比没有马芬综合症的妇女分娩的可能性更低(p <0.001)。没有孕产妇死亡。
结论:马凡氏综合征的女性更可能早产,SGA和剖宫产。未发现早产和SGA婴儿的这些增加的风险与他们的初生有关。患有已知胎儿马凡氏综合征的孕妇必须进行仔细监测。这些结果对于产科医生进行孕前咨询和治疗患有马凡氏综合症的妇女非常重要。需要进一步的研究来评估妊娠期间的风险以及妊娠对马凡氏综合症女性心血管状况的长期影响。

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