妊娠期急性A型夹层与马凡氏综合征。

IF 0.3 Q4 SURGERY Thoracic and Cardiovascular Surgeon Reports Pub Date : 2021-01-01 Epub Date: 2021-03-11 DOI:10.1055/s-0040-1722705
Christian Heim, Philipp P Müller, Michael Weyand, Frank Harig
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引用次数: 1

摘要

马凡氏综合征(MFS)是一种结缔组织疾病,由FBN-1基因突变引起,影响包括血管系统在内的多个器官系统。在怀孕期间,由于心血管系统的变化,患有MFS的妇女发生主动脉夹层的风险增加。病例描述我们报告一名39岁的女性,在第三次怀孕的第33周发生急性主动脉夹层,急需进行挽救生命的手术。对胎儿在子宫内进行计算机断层扫描三维重建。结论对疑似或确诊的MFS患者进行监测是必要的,需要采取跨学科的治疗和预防策略。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Acute Type A Dissection during Pregnancy with Marfan's Syndrome.

Background  Marfan's syndrome (MFS) is a connective tissue disorder, caused by a mutation in the FBN-1 gene and affecting multiple organ systems including the vascular system. During pregnancy, women with MFS have an increased risk of aortic dissection due to changes in the cardiovascular system. Case Description  We present a 39-year-old woman that suffered from an acute aortic dissection in week 33 of her third pregnancy who urgently required lifesaving surgery. Three-dimensional reconstruction of computed tomography scan was performed with unborn child in utero. Conclusion  Monitoring of patients with suspected or confirmed MFS appears essential and interdisciplinary treatment and prevention strategies are required.

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