马凡综合征患者的妊娠结局。
Pregnancy outcomes among women with Marfan syndrome.
机构信息
Department of Obstetrics and Gynecology, Bichat-Claude Bernard Hospital, Université Paris 7, AP-HP, Paris, France.
出版信息
Int J Gynaecol Obstet. 2013 Sep;122(3):219-23. doi: 10.1016/j.ijgo.2013.04.013. Epub 2013 Jun 28.
OBJECTIVE
To determine cardiac and obstetric outcomes among women with Marfan syndrome (MS) whose pregnancies were managed in accordance with the French national guidelines.
METHODS
A descriptive analysis was conducted for a prospective cohort of 18 women with MS who gave birth in the maternity unit of Bichat-Claude Bernard Hospital, Paris, France, between January 1, 1998, and May 31, 2011. The study hospital was the national referral center for MS and related diseases.
RESULTS
A total of 22 pregnancies were recorded among the study cohort. Of these, 21 were managed according to the national guidelines. One woman who was referred to the study hospital during late pregnancy was not managed according to the national guidelines; this patient experienced aortic dissection at 37 weeks. In the cohort, aortic diameter did not increase significantly during pregnancy. Vascular fetal growth restriction was observed in 7 (31.8 %) of the pregnancies. Cesarean delivery was planned for 17 (77.3%) of the pregnancies.
CONCLUSION
Risk of aortic dissection was low among a cohort of pregnant women with MS who were managed according to the French national guidelines.
目的
评估按照法国国家指南管理的马凡综合征（MS）女性患者的妊娠母婴结局。
方法
对法国巴黎比沙医院妇产科于 1998 年 1 月 1 日至 2011 年 5 月 31 日期间分娩的 18 例 MS 孕妇前瞻性队列进行描述性分析。研究医院是 MS 及相关疾病的国家转诊中心。
结果
在研究队列中记录了 22 例妊娠。其中 21 例按照国家指南管理。1 例在妊娠晚期转诊至研究医院的患者未按国家指南管理，该患者在 37 周时发生主动脉夹层。在该队列中，妊娠期间主动脉直径无明显增加。7 例（31.8%）妊娠出现胎儿血管生长受限。17 例（77.3%）妊娠计划行剖宫产。
结论
按照法国国家指南管理的 MS 妊娠女性患者发生主动脉夹层的风险较低。
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