Anesthesia in Parturients Presenting with Marfan Syndrome

Background: Pregnant women with Marian 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 tabor, which may be difficult due to the high prevalence of dural ectasia (DE) in these patients. Objectives: To evaluate the multidisciplinary management of MS patients during labor. Methods: Nineteen pregnant women (31 pregnancies) with MS were followed by a multi-disciplinary team (cardiologist, obstetrician, anesthesiologist) prior to delivery. Results:. 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. Conclusions: The optimal anesthetic strategy during tabor in MS patients should be decided by a multi-disciplinary team. Anesthetic complications due to DE were not encountered during neuraxial block.

Automated summary

Pregnant women with Marfan syndrome at high risk of aortic dissection around delivery require multidisciplinary management, including proper cardio-obstetric care and adequate pain management. Anesthetic complications due to dural ectasia were not encountered during neuraxial block.