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Management of a Parturient with Severe Aortic root Dilation and Complicated by Type A Aortic Dissection.
Abstract Number: T3C-7
Abstract Type: Case Report/Case Series
INTRODUCTION: Pregnancy associated with Marfan syndrome (MPS) leads to an increased risk of maternal/fetal complications; aortic dissection is the leading cause of morbidity and mortality. MPS often remains undiagnosed and is recognized only after life-threatening complications occurring during pregnancy or after delivery1.
CASE REPORT: A 23 y.o. G2P0 parturient at 27 wks presented with sudden-onset chest pain radiating to her back, associated with sweating. She was normotensive, with regular HR and CXR and EKG within normal limits. Her mother died age 35 from unknown cardiovascular disease. Physical exam revealed Marfanoid features. She underwent urgent TTE, showing an LVEF of 56%, severe aortic valve regurgitation, and a severely dilated (6.5 cm) ascending aorta. MRA was negative for aortic dissection. She was asymptomatic, hemodynamically stable and was admitted to cardiac ICU for continuous invasive monitoring. Esmolol infusion initiated to maintain HR<80 and SBP<120. After CT surgeon, obstetric, neonatology and anesthesiologists review a decision was taken to perform early CS. Interval repair and replacement of the aortic root was also planned. She received steroid prior to CS to facilitate fetal lung maturity. CSE with a small intrathecal dose was used for CS, supported by slow titration of epidural anesthesia. LUD placed to ensure adequate IVC flow. Oxytocin IM was given after delivery. She remained hemodynamically stable throughout. Four hours post delivery she developed chest pain but remained hemodynamically stable. Stat CTA showed type A aortic dissection with intimomedial flaps. A successful aortic valve and aortic root replacement was performed under GA. Post-bypass TEE showed normal LV and RV systolic function, normal grafts, and no aortic stenosis or regurgitation; neurology was intact. She was weaned from vasopressors on day 4 and discharged on POD 8.
DISCUSSION: Peripartum hemodynamic changes can be life-threatening to the parturient with MPS and thoracic aortic root dilation. Maternal mortality is 10% in those with an aortic root diameter > 4 cm. Our primary aim was to reduce the cardiovascular stress of delivery. We successfully managed the patient using anti-hypertensive medications and close monitoring of hemodynamical status during and immediate postpartum, using low dose CSE. Successful stabilization during delivery is critical to avoid emergency intrapartum CV surgery.
REFERENCE:1.Goland et al. Pregnancy and MPS; Ann CT Surg 2017