Cheulot P, Saucedo M, Bouvier-Colle M H, Deneux Tharaux C, Kayem G
Service de gynécologie-obstétrique, hôpital Armand-Trousseau, Assistance publique-Hôpitaux de Paris, 26, avenue du Dr Arnold-Netter, 75012 Paris, France.
Inserm U1153, équipe EPOPé, épidémiologie obstétricale périnatale et pédiatrique, centre de recherche en statistiques et épidémiologie, université Paris Descartes, DHU risques et grossesse, 53, avenue de l'observatoire, 75014 Paris, France.
Gynecol Obstet Fertil Senol. 2019 Jan;47(1):30-35. doi: 10.1016/j.gofs.2018.11.003. Epub 2018 Nov 27.
To describe maternal deaths in France associated with Marfan's syndrome or vascular Ehlers-Danlos syndrome.
A retrospective descriptive study based on data from the national confidential enquiry into maternal deaths, in France, during 2001-2012. Characteristics of the patients, their pregnancies and details of their deaths were analysed. The specific maternal mortality ratio by Marfan's syndrome or vascular Ehlers-Danlos syndrome was estimated.
Among 973 maternal deaths that occurred during the study period, five (0.4%) had a Marfan's syndrome (n=3) or a vascular Ehlers-Danlos syndrome (n=2), confirmed or suspected. The maternal mortality ratio due to Marfan's syndrome or vascular Ehlers-Danlos syndrome between 2001 and 2012 was 0.04/100,000 live births (IC 95% [0.011-0.2]). Three maternal deaths were caused by aortic dissections and two by other arterial ruptures. The deaths have occurred after 37 weeks of pregnancy for 4 patients, and at fifteen days of post-partum for one patient. The median age of death was 30 years. Three patients were nulliparous. Marfan's syndrome and vascular Ehlers-Danlos syndrome were not identified before the death of these five patients.
Five patients with, or suspected to have, Marfan's syndrome or vascular Ehlers-Danlos syndrome were identified. Early diagnosis of these syndromes in pregnant women before life threatening events is very important, especially to refer them to appropriate care.
描述法国与马凡综合征或血管性埃勒斯-当洛综合征相关的孕产妇死亡情况。
一项基于2001 - 2012年法国全国孕产妇死亡保密调查数据的回顾性描述性研究。分析了患者特征、妊娠情况及其死亡细节。估算了马凡综合征或血管性埃勒斯-当洛综合征的特定孕产妇死亡率。
在研究期间发生的973例孕产妇死亡中，有5例（0.4%）确诊或疑似患有马凡综合征（n = 3）或血管性埃勒斯-当洛综合征（n = 2）。2001年至2012年期间，马凡综合征或血管性埃勒斯-当洛综合征导致的孕产妇死亡率为0.04/10万活产（95%可信区间[0.011 - 0.2]）。3例孕产妇死亡由主动脉夹层引起，2例由其他动脉破裂引起。4例患者在妊娠37周后死亡，1例在产后15天死亡。死亡的中位年龄为30岁。3例患者为初产妇。这5例患者在死亡前均未被诊断出马凡综合征和血管性埃勒斯-当洛综合征。
共识别出5例患有或疑似患有马凡综合征或血管性埃勒斯-当洛综合征的患者。在危及生命的事件发生前对孕妇进行这些综合征的早期诊断非常重要，尤其是要将她们转诊至适当的医疗机构。
