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妊娠合并马方氏综合征分娩时发生主动脉夹层1例报告

A Case of Acute Aortic Dissection Following Intrapartum in a Patient with Marfan ’ s Syndrome

  • 摘要: 患者,女,33岁,G4P1+2,以“胚胎移植术后34+4周,皮肤瘙痒5+ d”入院。2+年前该患者因马方氏综合征行第三代试管婴儿,足月顺产一女活婴。此次患者孕期产检时糖耐量实验提示妊娠期糖尿病,经糖尿病医学营养管理及运动控制血糖后,血糖波动于正常范围;孕期心脏超声检查发现主动脉窦部增宽至40 mm,左室收缩功能测值正常。入院前5+ d,患者确诊为“妊娠期肝内胆汁淤积症(轻度)”,入院后根据胚胎移植时间核实孕周为37+2周,修正诊断“妊娠期肝内胆汁淤积症(重度)”。缩宫素激惹试验过程中患者自诉腰背部疼痛,考虑主动脉夹层可能,多学科合作下行剖宫产术,术中行双侧子宫动脉结扎,分娩一活男婴,体质量3 020 g,Apgar评分(娩出后1-5-10 min)为10-10-10分,术中出血量共600 mL。术后行CT血管造影示主动脉夹层动脉瘤(B型)。随访至今,患者无胸闷气促等不适,产后恢复好。

     

    Abstract: A 33-year-old woman was admitted at 34+4 weeks after embryo transfer and over 5 d of cutaneous pruritus. Over 2 years ago, the patient had a third-generation test tube baby due to Marfan's syndrome and gave birth to a live baby girl at full term. This time, the glucose tolerance test during the prenatal examination indicated that the patient was with gestational diabetes, and the blood glucose fluctuated within the normal range after the medical nutrition management and exercise control. The heart ultrasound examination during pregnancy showed that the aortic sinus was enlarged to 40 mm, and the left ventricular systolic function was normal. The patient was diagnosed as "intrahepatic cholestasis of pregnancy (mild)". After admission, the gestational age was verified to be 37+2 weeks according to the time of embryo transfer, and the diagnostic result was modified to be intrahepatic cholestasis of pregnancy (severe). During the test, the patient complainted back pain, which might interpreted as a sign of aortic dissection. Cesarean section was carried out under multidisciplinary cooperation, during which the amount of bleeding was 600 mL, bilateral uterine artery ligation was performed and a live baby boy was delivered. The body mass of the baby was 3 020 g, and the Apgar scores (1-5-10 min after delivery) were 10-10-10. The postoperative CT angiography showed aortic dissection (Type B). It was found in the follow-up that the patient had no discomforts such as chest tightness and shortness of breath, and recovered well after the delivery.

     

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