An Unusual Case of a Parturient with Uncorrected Pentalogy of Fallot Presenting for Elective Cesarean Section Delivery of Twins

We present a 31-year-old primigravida with uncorrected pentalogy of Fallot, pregnant with monochorionic-diamniotic twins, undergoing elective lower segment cesarean section at 36 weeks gestation. Preoperative workup included a transthoracic echocardiogram which revealed a large ventricular septal defect of 1.8 cm with bidirectional shunting, a moderate size atrial septal defect of 1.8 cm with predominant left-to-right shunting, an overriding aorta, moderate right ventricular hypertrophy, and severe pulmonary valve stenosis. Notably, the patient was acyanotic with normal effort tolerance. Preoperative preparation involved the input of cardiologists and obstetric and cardiothoracic anesthetists. Issues such as the use of extracorporeal membrane oxygenation and cardiopulmonary support in the event of cardiac failure were discussed. Autotransfusion postdelivery was also addressed, and plans made for therapeutic venesection should need to arise. Intraoperatively, the planned anesthetic technique was slow and titrated combined spinal-epidural. However, a general anesthetic technique with rapid sequence induction was used in view of extreme patient anxiety. Intravenous induction was performed with ketamine and etomidate, followed by paralysis with succinylcholine. Anesthesia was maintained with desflurane on a mixture of air and oxygen. Phenylephrine infusion was titrated according to the patient's blood pressure and systemic vascular resistance. The uterotonic of choice was duratocin given as a slow bolus, followed by a 4-h infusion of oxytocin. The patient was put in a head-up position to prevent venous air embolism and to decrease autotransfusion to central circulation. Postoperatively, she was extubated and sent to the Intensive Care Unit for continuous monitoring with FloTrac®.