Postpartum Stanford type B aortic dissection in a woman with Loeys-Dietz syndrome who underwent a prophylactic aortic root replacement before conception: A case report
Postpartum Stanford type B aortic dissection in a woman with Loeys-Dietz syndrome who underwent a prophylactic aortic root replacement before conception: A case report
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DOI：
10.1016/j.tjog.2020.10.005
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发表时间：
2021-01-01
影响因子：
2.1
通讯作者：
Fujii, Tomoyuki
中科院分区：
文献类型：
--
作者：
Shitara, Rieko;Iriyama, Takayuki;Fujii, Tomoyuki
目的：Loeys-Dietz综合征（LDS）与妊娠期和产后发生主动脉夹层（AD）的风险较高相关。然而，有有限的证据表明，在怀孕前已经接受预防性主动脉根部置换术（ARRs）的LDS患者的围产期管理。病例报告：我们提出的情况下，28岁的未经生产的孕妇与LDS与致病性变异内的外显子5的TGFBR 2（c.1379G > T，p. [Arg 460 Leu]），在20岁时接受了ARR。心脏超声心动图未显示妊娠期间主动脉有任何显著变化，血压保持正常。她在怀孕37周时进行了剖腹产。她在产后8天发生急性斯坦福大学B型AD，从主动脉弓延伸到肾下主动脉，并接受了全弓置换术。结论：该病例报告表明，预防性ARR后的LDS患者仍有发生斯坦福大学B型AD的风险。? 2021台湾妇产科学会Taiwan Association of Obstetrics & Gynecology Elsevier B. V.的出版服务。这是一篇在CC BY-NC-ND许可证下的开放获取文章（http：//creativecommons.org/licenses/by-nc-nd/4.0/）。
Objective: Loeys-Dietz syndrome (LDS) is associated with a higher risk of aortic dissections (ADs) during pregnancy and postpartum. However, there is limited evidence about the perinatal management of LDS patients who have undergone prophylactic aortic root replacements (ARRs) before conception. Case report: We present the case of a 28-year-old nulliparous pregnant woman with LDS with a pathogenic variant within exon 5 of TGFBR2 (c.1379G > T, p.[Arg460Leu]), who underwent an ARR at 20 years of age. Cardiac echocardiography did not show any significant changes in the aorta during pregnancy, and her blood pressure remained normal. She had a cesarean section at 37 weeks of gestation. She developed an acute Stanford type B AD extending from the aortic arch to the infrarenal aorta 8 days postpartum and underwent a total arch replacement. Conclusion: This case report suggests that patients with LDS after prophylactic ARRs still possess a risk for Stanford type B ADs. ? 2021 Taiwan Association of Obstetrics & Gynecology. Publishing services by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).