Department of Anesthesiology, Sheba Medical Center, Tel Hashomer, Israel.
Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Isr Med Assoc J. 2021 Jul;23(7):437-440.
Pregnant women with Marfan syndrome (MS) have a high risk of aortic dissection around delivery and their optimal management requires a multi-disciplinary approach, including proper cardio-obstetric care and adequate pain management during labor, which may be difficult due to the high prevalence of dural ectasia (DE) in these patients.
To evaluate the multidisciplinary management of MS patients during labor.
Nineteen pregnant women (31 pregnancies) with MS were followed by a multi-disciplinary team (cardiologist, obstetrician, anesthesiologist) prior to delivery.
Two patients had kyphoscoliosis; none had previous spine surgery nor complaints compatible with DE. In eight pregnancies (7 patients), aortic root diameter (ARd) before pregnancy was 40 to 46 mm. In this high-risk group, one patient underwent elective termination, two underwent an urgent cesarean section (CS) under general anesthesia, and five had elective CS; two under general anesthesia (GA), and three under spinal anesthesia. In 23 pregnancies (12 patients), ARd was < 40 mm. In this non-high-risk group three pregnancies (1 patient) were electively terminated. Of the remaining 20 deliveries (11 patients), 14 were vaginal deliveries, 9 with epidural analgesia and 5 without. Six patients had a CS; four under GA and two2 under spinal anesthesia. There were no epidural placement failures and no failed responses. There were 2 cases of aortic dissection, unrelated to the anesthetic management.
The optimal anesthetic strategy during labor in MS patients should be decided by a multi-disciplinary team. Anesthetic complications due to DE were not encountered during neuraxial block.
患有马凡综合征（Marfan syndrome，MS）的孕妇在分娩前后发生主动脉夹层的风险较高，其最佳管理需要多学科方法，包括适当的心脏产科护理和分娩时充分的疼痛管理，但由于这些患者硬膜扩张症（dural ectasia，DE）的高患病率，可能会存在一定难度。
评估多学科团队在 MS 患者分娩期间的管理方法。
19 名孕妇（31 次妊娠）在分娩前由多学科团队（心脏病专家、产科医生、麻醉师）进行随访。
2 名患者存在脊柱后凸侧凸；无既往脊柱手术史，也无符合 DE 的症状。8 次妊娠（7 名患者）中，主动脉根部直径（aortic root diameter，ARd）在妊娠前为 40 至 46mm。在这一高危组中，1 名患者选择了择期终止妊娠，2 名患者在全身麻醉下紧急行剖宫产术（cesarean section，CS），5 名患者选择了择期 CS；2 名在全身麻醉下，3 名在脊髓麻醉下。23 次妊娠（12 名患者）中，ARd＜40mm。在这一非高危组中，3 次妊娠（1 名患者）选择了择期终止妊娠。20 次分娩（11 名患者）中，14 次为阴道分娩，9 次使用硬膜外镇痛，5 次未使用。6 名患者行 CS；4 名在全身麻醉下，2 名在脊髓麻醉下。没有硬膜外置管失败，也没有镇痛失败。有 2 例主动脉夹层，与麻醉管理无关。
多学科团队应决定 MS 患者分娩时的最佳麻醉策略。在脊髓麻醉中未遇到 DE 引起的麻醉并发症。
