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一名患有马凡综合征的患者在妊娠28周时发生急性主动脉夹层,接受了主动脉根部置换术治疗。

A patient with Marfan's syndrome who developed an acute aortic dissection at 28 weeks of pregnancy treated with aortic root replacement.

作者信息

Sumiyoshi Riki, Morita Hideki, Fujii Kento, Shirasughi Takehiro, Kawaura Hiroyuki, Aoki Masakazu, Nagano Hiroshi

机构信息

Department of Cardiovascular Surgery, Saitama Red Cross Hospital, 1-5 Shintoshin, Chuo-ku, Saitama-shi, Saitama, 330-8553, Japan.

出版信息

Gen Thorac Cardiovasc Surg. 2021 Jun;69(6):1004-1007. doi: 10.1007/s11748-021-01596-3. Epub 2021 Feb 7.

DOI:10.1007/s11748-021-01596-3
PMID:33550484
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8131282/
Abstract

The patient was a 31-year-old pregnant woman who gave birth to her first child by vaginal delivery 7 years ago. She was diagnosed with Marfan's syndrome based on physical findings; however, the condition was not diagnosed before the onset. The patient developed acute aortic dissection at 28 weeks of pregnancy. A cesarean section was first performed to save the patient's life; then, a total hysterectomy was performed to prevent the risk of postpartum hemorrhage. Furthermore, aortic root replacement was performed using a temporary mechanical valve. The patient and her child have survived without any complications.

摘要

该患者为一名31岁的孕妇,7年前经阴道分娩产下她的第一个孩子。根据体格检查结果,她被诊断为马凡综合征;然而,在发病前该疾病未被诊断出来。该患者在怀孕28周时发生了急性主动脉夹层。首先进行剖宫产以挽救患者生命;然后,进行全子宫切除术以防止产后出血的风险。此外,使用临时机械瓣膜进行了主动脉根部置换术。患者及其孩子均存活且无任何并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6c3d/8131282/b3219512d9a8/11748_2021_1596_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6c3d/8131282/3c947272ff0b/11748_2021_1596_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6c3d/8131282/de3c369726a2/11748_2021_1596_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6c3d/8131282/b3219512d9a8/11748_2021_1596_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6c3d/8131282/3c947272ff0b/11748_2021_1596_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6c3d/8131282/de3c369726a2/11748_2021_1596_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6c3d/8131282/b3219512d9a8/11748_2021_1596_Fig3_HTML.jpg

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本文引用的文献

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Exp Ther Med. 2018 Dec;16(6):4407-4414. doi: 10.3892/etm.2018.6761. Epub 2018 Sep 19.
2
Vascular smooth muscle cells in Marfan syndrome aneurysm: the broken bricks in the aortic wall.马凡综合征动脉瘤中的血管平滑肌细胞:主动脉壁中的破碎砖块。
Cell Mol Life Sci. 2017 Jan;74(2):267-277. doi: 10.1007/s00018-016-2324-9. Epub 2016 Aug 17.
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先天性心脏病患者的妊娠问题。
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Acute aortic dissection at 33 weeks of gestation with fetal distress syndrome.妊娠33周时发生急性主动脉夹层并伴有胎儿窘迫综合征。
Gen Thorac Cardiovasc Surg. 2011 Aug;59(8):566-8. doi: 10.1007/s11748-010-0735-3. Epub 2011 Aug 18.
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Pregnancy-associated aortic dilatation or dissection in Japanese women with Marfan syndrome.马凡综合征日本女性妊娠相关主动脉扩张或夹层。
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