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Management of a Pregnant Patient with Scimitar Syndrome Presenting for Cesarean Delivery
Abstract Number: T-62
Abstract Type: Case Report/Case Series
Patient History: A 40-year-old G3P2 with Scimitar syndrome presented for a primary cesarean delivery (CD) after myomectomy. She had a medical history significant for increased Factors XI and XII requiring anticoagulation. The patient had two uneventful vaginal deliveries prior to her cardiopulmonary diagnosis and myomectomy. The CXR demonstrated a hypoplastic right lung and the cardiac MR showed anomalous drainage of right upper pulmonary vein to the right atrium via a ‘Scimitar’ vein. Echocardiogram demonstrated a moderately dilated left and right ventricle, preserved systolic function, with elevated RVSP.
Operative Course: She underwent uneventful CD and bilateral tubal ligation. A spinal was performed at the L3-4 interspace; however, this provided an inadequate surgical level and a combined spinal-epidural was then placed at L4-5. She was hemodynamically stable throughout.
Discussion: Scimitar syndrome is a congenital anomaly characterized by a partial anomalous pulmonary venous return (PAPVR) from the right lung into the IVC associated with right lung hypoplasia and cardiac dextroposition. Infants present with pulmonary hypertension or heart failure; adults are often asymptomatic. The shadow of the anomalous right pulmonary vein gives the appearance of a Turkish sword or “scimitar” on CXR; this “scimitar sign” is diagnostic and can be confirmed by MR angiography.
Normal physiologic changes of pregnancy, including increased total blood volume and cardiac output with decreased SVR and PVR have important implications. Our goals were to maintain hemodynamic stability, minimize shunting and prevent worsening of the patient’s pulmonary hypertension. Pain, hypoxemia, and hypercarbia were therefore avoided. Monitoring volume status was a priority as the pulmonary veins from the right lung draining into the IVC creates a left to right shunt, placing patients at risk of right heart failure secondary to volume overload. Moreover, during delivery there are increased fluid shifts secondary to blood loss, fluid administration, and uterine contraction causing auto-transfusion of blood back into systemic circulation.
Hendrie M, Mathur D. Scimitar syndrome in pregnancy. Indian J Anaesth. 2014; 58(2):208-210.
Tavare A, Wylie P. Scimitar syndrome. Q J Med. 2014; 107:477.
Yuksel M, Imamoglu M, Cakmak B, Oncl M, Madazli R. Scimitar syndrome and pregnancy, complicated with severe preeclampsia. Arch Gynecol Obstet. 2014; 289:1371-1373.