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马凡综合征与二叶式主动脉瓣疾病:产科结局及妊娠相关即刻和长期主动脉并发症的对比分析

Marfan Syndrome Versus Bicuspid Aortic Valve Disease: Comparative Analysis of Obstetric Outcome and Pregnancy-Associated Immediate and Long-Term Aortic Complications.

作者信息

Toprak Betül, Szöcs Katalin, Zengin-Sahm Elvin, Sinning Christoph, Hot Amra, Bannas Peter, Hecher Kurt, Hüneke Bernd, Mir Thomas S, Rybczynski Meike, Girdauskas Evaldas, Blankenberg Stefan, von Kodolitsch Yskert

机构信息

Department of Cardiology, University Heart and Vascular Center Hamburg, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany.

Department of Medical Biometry and Epidemiology, University Medical Center Hamburg-Hospital Eppendorf, 20246 Hamburg, Germany.

出版信息

J Clin Med. 2020 Apr 15;9(4):1124. doi: 10.3390/jcm9041124.

DOI:10.3390/jcm9041124
PMID:32326432
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7230569/
Abstract

Pregnancy poses a threat to women with aortopathy. Conclusive data on the obstetric and aortic outcome in this risk collective, especially when it comes to aortic complications in the long term, are still missing. This study offers a comparative analysis of pregnancy-associated outcome in 113 consecutive women with Marfan syndrome or bicuspid aortic valve disease, including 46 ever-pregnant and 37 never-pregnant women with Marfan syndrome, and 23 ever-pregnant and 7 never-pregnant females with bicuspid aortic valve disease. The overall obstetric outcome was comparable between ever-pregnant women with Marfan syndrome and with bicuspid aortic valve disease ( = 0.112). Pregnancy-associated aortic dissection occurred in two women with Marfan syndrome (3%) during a total of 62 completed pregnancies, whereas no single case of aortic event occurred in women with bicuspid aortic valve disease during a total of 36 completed pregnancies ( = 0.530). In the long-term follow-up, aortic dissection occurred in 21% of ever-pregnant women with Marfan syndrome, but in none of the women with bicuspid aortic valve disease ( 0.022). Proximal aortic surgery was performed with similar frequency in ever-pregnant women with Marfan syndrome and with bicuspid aortic valve disease in the long term ( = 0.252). However, ever-pregnant women with Marfan syndrome were younger when surgery was performed (44 ± 9 vs. 59 ± 7 years; = 0.041). In Marfan syndrome, long-term growth of the aorta was comparable between ever-pregnant and never-pregnant women. Pregnancy thus exhibited an increased immediate aortic risk only in women with Marfan syndrome, but not in women with bicuspid aortic valve disease. Previous pregnancy did not relate to an increased long-term risk of adverse aortic events in women with Marfan syndrome or with bicuspid aortic valve disease.

摘要

妊娠对患有主动脉病变的女性构成威胁。关于这一风险群体的产科和主动脉结局的确定性数据仍然缺失,尤其是长期的主动脉并发症方面。本研究对113例连续的患有马凡综合征或二叶式主动脉瓣疾病的女性的妊娠相关结局进行了比较分析,其中包括46例有过妊娠经历和37例从未妊娠的马凡综合征女性,以及23例有过妊娠经历和7例从未妊娠的二叶式主动脉瓣疾病女性。有过妊娠经历的马凡综合征女性和二叶式主动脉瓣疾病女性的总体产科结局相当(P = 0.112)。在总共62次完整妊娠期间,2例患有马凡综合征的女性(3%)发生了妊娠相关主动脉夹层,而在总共36次完整妊娠期间,患有二叶式主动脉瓣疾病的女性未发生任何主动脉事件(P = 0.530)。在长期随访中,21%有过妊娠经历的马凡综合征女性发生了主动脉夹层,但二叶式主动脉瓣疾病女性均未发生(P = 0.022)。从长期来看,有过妊娠经历的马凡综合征女性和二叶式主动脉瓣疾病女性进行升主动脉手术的频率相似(P = 0.252)。然而,进行手术时,有过妊娠经历的马凡综合征女性更年轻(44±9岁 vs. 59±7岁;P = 0.041)。在马凡综合征中,有过妊娠经历和从未妊娠的女性主动脉的长期生长情况相当。因此,妊娠仅在马凡综合征女性中表现出增加的主动脉近期风险,而在二叶式主动脉瓣疾病女性中则没有。既往妊娠与马凡综合征或二叶式主动脉瓣疾病女性发生不良主动脉事件的长期风险增加无关。

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