Anesthesia in Parturients Presenting with Marfan Syndrome

Isr Med Assoc J. 2021 Jul;23(7):437-440.

Abstract

Background: 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.

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 labor in MS patients should be decided by a multi-disciplinary team. Anesthetic complications due to DE were not encountered during neuraxial block.

MeSH terms

  • Adult
  • Anesthesia, Obstetrical* / adverse effects
  • Anesthesia, Obstetrical* / methods
  • Anesthesia, Obstetrical* / statistics & numerical data
  • Aortic Diseases* / complications
  • Aortic Diseases* / diagnosis
  • Aortic Diseases* / etiology
  • Aortic Dissection* / diagnosis
  • Aortic Dissection* / prevention & control
  • Delivery, Obstetric* / adverse effects
  • Delivery, Obstetric* / methods
  • Delivery, Obstetric* / statistics & numerical data
  • Female
  • Humans
  • Interdisciplinary Communication
  • Israel / epidemiology
  • Marfan Syndrome* / complications
  • Marfan Syndrome* / epidemiology
  • Marfan Syndrome* / physiopathology
  • Monitoring, Physiologic / methods
  • Obstetric Labor Complications* / diagnosis
  • Obstetric Labor Complications* / etiology
  • Obstetric Labor Complications* / prevention & control
  • Outcome and Process Assessment, Health Care
  • Pregnancy
  • Pregnancy Complications* / diagnosis
  • Pregnancy Complications* / physiopathology
  • Pregnancy Complications* / therapy
  • Pregnancy Outcome / epidemiology
  • Pregnancy, High-Risk