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Anesthetic Management of Marfan Syndrome in Parturients
Abstract Number: T-10
Abstract Type: Case Report/Case Series
Introduction: Marfan syndrome is an autosomal dominant connective tissue disorder that can predispose patients to aortic root dilation and risk of aortic dissection. Parturients with Marfan syndrome with an aortic root diameter < 4 cm typically tolerate pregnancy well, although the hemodynamic challenges of labor and delivery pose substantial risk for cardiovascular complications. For parturients allowed a trial of vaginal delivery, neuraxial techniques during labor may help to lower risk of dissection. However there is concern regarding the existence of dural ectasia in this patient population and risk of cerebrospinal fluid (CSF) leak in cases of inadvertent dural puncture (1,2). For Marfan patients undergoing cesarean delivery, the potential for dural ectasia causing erratic spread of spinal anesthesia is a concern. The optimal mode of analgesia and anesthesia for parturients with Marfan syndrome is not clearly established.
Methods: We retrospectively evaluated the anesthetic management of parturients with Marfan syndrome who delivered at our institution during the past six years.
Results: Six pregnancies in 5 parturients were identified. Four of 6 cases (67%) had an antepartum anesthesia consultation. The planned mode of delivery was vaginal for 3 of the pregnancies which resulted in 2 vaginal deliveries in the same patient and another cesarean delivery for failure to progress with epidural analgesia/anesthesia. Three more cesarean deliveries were performed; 2 for dilated aortic root diameter, and 1 for active herpes simplex virus lesions. Of the 4 cesarean deliveriess that were carried out, 2 were performed under spinal anesthesia and 2 were under epidural anesthesia. One cerclage placement was performed under spinal anesthesia. All epidural and spinal anesthetics provided adequate analgesia/anesthesia. Four of the parturients had aortic root diameter < 4 cm (2.6 - 3.96 cm); two had aortic root diameter >4 cm (4.15 - 4.2 cm). All of the births occurred at term gestation. Only one parturient had previous imaging of the spine, which did not show any evidence of dural ectasia. Four parturients were managed on beta blocker; one refused. No aortic dissection or other complications occurred during their pregnancy or postpartum.
Discussion: Early literature on Marfan syndrome in pregnancy recommended general over spinal anesthesia for cesarean delivery due to the high prevalence of dural ectasia in this patient population (63-92%) (2,3). However, the use of combined spinal epidural techniques have more recently been advocated to mitigate hemodynamic shifts (2). Our experience managing 6 pregnancies in patients with Marfan syndrome further validate the use of neuraxial techniques for vaginal and cesarean delivery in women with Marfan syndrome.
1. Fattori, et al. Lancet 1999
2. Kim G, et al. Korean J Anesthesiol 2011
3. Allyn, et al. Anesth Analg 2013