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Delivery of the parturient with Marfan syndrome following aortic root replacement and multiple spinal fusions
Abstract Number: SU-99
Abstract Type: Case Report/Case Series
Introduction: Marfan syndrome presents a number of challenges in the parturient, with implications involving cardiovascular function and spinal anatomy among others.
Case Presentation: A 31 year-old nulliparous woman at 37 2/7 weeks EGA presented to our labor and delivery unit for primary cesarean delivery secondary to breech position. Past medical history was significant for Marfan syndrome. Surgical history included valve-sparing aortic root replacement, pes excavatum repair, spinal fusion and reoperation, and intraocular lens replacement. She reported excellent functional capacity, demonstrating 6-10 metabolic equivalents without dyspnea. BMI at term was 36.6 kg/m2. Airway exam was reassuring. Cardiac exam revealed Grade III/VI systolic heart murmur at right upper sternal border. CTA performed one year prior to pregnancy revealed an aortic root diameter of 3.3 cm and midthoracic decending aorta of 2.1 cm. 2DE performed during her 3rd trimester revealed aortic root diameter of 3.5 cm, normal LV function, and mild to moderate AI. On presentation, a left radial arterial line was placed for close hemodynamic monitoring. After 2 failed epidural catheters, the block was allowed time to wear off and subsequently spinal anesthesia was performed and adequate surgical level was achieved. Cesarean delivery proceeded without incident and a healthy male infant with Apgar scores of 9/9 was delivered. Her postopertive course was uneventful; both mom & baby were doing well at 3 month follow up.
Discussion: Marfan syndrome is an autosomal dominant connective tissue disorder with prevalence estimated at 1:10,000. This diagnosis presents a number of anesthetic implications in the parturient, with risk for pectus deformity, difficult airway management due to high arched palate and cervical spine pathology, kyphoscoliosis with or without prior surgical repair, cardiovascular complications including risk of aortic dissection and rupture.
80% of parturients with Marfan syndrome have cardiac involvement, with the greatest risk for morbidity and mortality being secondary to aortic aneurysm rupture and dissection. Although estimated risk of cardiovascular complications in parturients with aortic rood diameter < 40 mm is 1%, this risk is less clear in the parturient with history of aortic root replacement. Regardless of the risk, careful hemodynamic monitoring and management is imperative in this patient population, and slow induction of neuraxial anesthesia via epidural or sequential CSE may be preferred. Scoliosis is reported in 60% of patients, with up to 50% requiring surgical correction. Further, dural ectasia is more common in this patient population. These parturients who have undergone prior spinal fusion therefore may present a challenge for both epidural and spinal anesthesia. Multidisciplinary planning is crucial for risk stratification and delivery planning for this patient population.