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一名患有马凡综合征的产后患者发生B型主动脉夹层破裂。

Rupture of a Type B Aortic Dissection in a Postpartum Patient with Marfan Syndrome.

作者信息

Patberg Elizabeth, Duffy Jennifer, Hameed Afshan B

机构信息

Department of Obstetrics and Gynecology, Irvine Medical Center, University of California, Orange, California.

出版信息

AJP Rep. 2019 Jul;9(3):e256-e261. doi: 10.1055/s-0039-1692712. Epub 2019 Aug 20.

Abstract

Pregnant women with Marfan syndrome (MFS) are known to be at increased risk of aortic dissection; however, cases of aortic rupture are extremely rare. There is lack of consensus on the exact site and size of aortic diameter measurement that increases this risk, and whether this applies to both Type A and Type B dissections.  A 23-year-old G2P1001 with known Marfan syndrome who underwent an uncomplicated antepartum and intrapartum course. She experienced persistent backache 10 days postpartum that led to the diagnosis of Stanford Type B dissection. The patient was hospitalized for close observation. Dissection progressed to aortic rupture within 24 hours that required emergent thoracic endovascular aortic repair. She had an uncomplicated postoperative course.  Our report demonstrates rupture of a known aortic dissection within a very short time in the postpartum period. The case highlights the importance of patient education and close surveillance especially in the postpartum period. It also brings home the value of imaging of the whole aorta rather than focusing on the ascending alone. Multidisciplinary care and timely diagnosis and intervention likely led to the favorable outcome in our case.

摘要

已知患有马凡综合征(MFS)的孕妇发生主动脉夹层的风险增加;然而,主动脉破裂的病例极为罕见。对于增加这种风险的主动脉直径测量的确切部位和大小,以及这是否适用于A型和B型夹层,目前尚无共识。一名23岁、孕2产1(G2P1001)且已知患有马凡综合征的孕妇,产前和产程均无并发症。产后10天她持续背痛,随后被诊断为斯坦福B型夹层。患者住院密切观察。夹层在24小时内进展为主动脉破裂,需要紧急进行胸段血管腔内主动脉修复术。她术后恢复顺利。我们的报告显示,已知的主动脉夹层在产后很短时间内发生破裂。该病例突出了患者教育和密切监测的重要性,尤其是在产后阶段。它还强调了对整个主动脉进行成像的价值,而不仅仅关注升主动脉。多学科护理以及及时的诊断和干预可能是我们这个病例取得良好结果的原因。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/16c1/6702027/69f06b8ba30f/10-1055-s-0039-1692712-i190031-1.jpg

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