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Management of a Parturient with Scimitar Syndrome
Abstract Number: RF7B10-240
Abstract Type: Case Report Case Series
Background: Scimitar syndrome is a rare congenital cardiopulmonary anomaly occurring in 1/100,000 births, which include partial or total anomalous pulmonary venous connection from the right lung to the inferior vena cava, right lung hypoplasia which causes dextrocardia or dextroposition of the heart, and variable systemic arterial right lung blood supply. The anomalous pulmonary venous return causes a left-to-right shunt and is most commonly associated with an atrial septal defect.  The adult form tends to be asymptomatic or carries vague symptoms of fatigue, dyspnea and recurrent upper respiratory infections. The infantile form is characterized by severe tachypnea, failure to thrive, and cyanosis that is typically coupled with pulmonary hypertension. 
Case: A 34 year old G1P0 was evaluated by cardiology at 37w3d for shortness of breath and orthopnea. A TTE revealed an EF of 70%, a dilated RA and RV, and atrial shunting. CXR was notable for a positive Scimitar sign indicating anomalous pulmonary venous return, as well as dextrocardia vs. dextroposition. EKG indicated right superior axis deviation, incomplete RBBB, and pulmonary disease pattern. The cardiology team diagnosed the patient with Scimitar syndrome and cleared the patient for a normal vaginal delivery. The patient presented to L&D at 40w5d with complaints of increased shortness of breath and 10 pillow orthopnea. The obstetric workup was diagnostic of severe preeclampsia and, therefore, induction of labor was initiated. An epidural was placed uneventfully for labor analgesia. A repeat TTE, CXR, and EKG were obtained. TTE showed an EF of 60-65% and was otherwise unchanged from her previous echo. CXR revealed signs of mild congestive heart failure and increased vasculature of the lower lobes. EKG remained unchanged. The patient was taken for a cesarean section due to an arrested labor course. Surgical anesthesia was unobtainable via the epidural catheter which resulted in conversion to general anesthesia. The cesarean section was complicated by increased blood loss due to uterine atony. Post-operatively, the patient became hypotension and tachycardic. She was evaluated by the obstetric team and was found to have post-partum hemorrhage, with an estimated blood loss of 1750ml. Two units PRBC’s was administered. The patient’s post-operative course was complicated by pulmonary edema requiring diuresis and severe orthopnea. She was discharged on POD #5.
Conclusion: Patients with Scimitar syndrome who survive into adulthood are usually well compensated and are often asymptomatic.  However, with the increase in intravascular volume associated with pregnancy, the incidence of congestive heart failure and volume overload increases.  It is possible for women with Scimitar syndrome to have an uncomplicated pregnancy and delivery. However, it is important to be aware of the risks and anesthetic implications associated maternal Scimitar syndrome.