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

Taiwanese Journal of Obstetrics & Gynecology

Tomoyuki Fujii¹  Seisuke Sayama²  Takayuki Iriyama³  Yutaka Osuga³  Norifumi Takeda⁴  Takeshi Nagamatsu⁵  Rieko Shitara⁵ 

[1] Corresponding author. Department of Obstetrics &;Departments of Obstetrics &Gynecology, Faculty of Medicine, University of Tokyo, Tokyo, Japan;Gynecology, Japan Community Health Care Organization Tokyo Shinjuku Medical Center, Tokyo, Japan;;Departments of Obstetrics &

关键词: Aortic dissection;    Aortic root replacement;    Loeys-Dietz syndrome;    Postpartum period;    Pregnancy;   

DOI  :  

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown