妊娠前接受预防性主动脉根部置换术的洛伊丝-迪茨综合征女性患者发生产后斯坦福 B 型主动脉夹层：1 例报告。
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.
机构信息
Departments of Obstetrics & Gynecology, Faculty of Medicine, University of Tokyo, Tokyo, Japan; Departments of Obstetrics & Gynecology, Japan Community Health Care Organization Tokyo Shinjuku Medical Center, Tokyo, Japan.
Departments of Obstetrics & Gynecology, Faculty of Medicine, University of Tokyo, Tokyo, Japan.
出版信息
Taiwan J Obstet Gynecol. 2021 Jan;60(1):145-147. doi: 10.1016/j.tjog.2020.10.005.
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.
目的
Loeys-Dietz 综合征（LDS）与妊娠和产后主动脉夹层（AD）的风险增加相关。然而，对于在妊娠前已行预防性主动脉根部置换术（ARR）的 LDS 患者的围产期管理，证据有限。
病例报告
我们报告了一例 28 岁的初产妇 LDS 病例，携带 TGFBR2 外显子 5 内的致病性变异（c.1379G>T，p.[Arg460Leu]），她在 20 岁时接受了 ARR。妊娠期间心脏超声心动图未显示主动脉有任何明显变化，其血压保持正常。她于孕 37 周行剖宫产术。产后 8 天，她发生急性 Stanford 型 B 型 AD，从主动脉弓延伸至肾下主动脉，行全主动脉弓置换术。
结论
本病例报告提示预防性 ARR 后 LDS 患者仍存在 Stanford 型 B 型 AD 的风险。
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