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Case presentation
A 21-year-old, gravida 1, para 0, presented at 30 weeks of gestation after having tried to conceive unsuccessfully for 1.5 years. The patient was born with pulmonary atresia for which she underwent a modified Blalock-Taussig shunt in infancy, right ventricular (RV) outflow tract reconstruction at 21 months, and atrial septal defect closure with further reconstruction of the RV outflow tract and a side-to-side connection of the superior vena cava to the right pulmonary artery at 6 years. Cardiac physiology was described as classic Fontan.
Three years before pregnancy, cardiac evaluation showed systemic venous pressures of 15 mm Hg and left ventricular ejection fraction (LVEF), 33%. Echocardiography at 30 weeks of gestation confirmed the above anatomy as well as Ebstein anomaly of the tricuspid valve, a markedly dilated right atrium, and a hypoplastic RV. Holter monitoring revealed frequent self-limited episodes of supraventricular tachycardia that was clinically associated with symptoms ranging from fluttering to chest pain without syncope. The patient was started on metoprolol at approximately 3 months of gestation with the addition of amiodarone at approximately 7 months of gestation. Shortness of breath and lower extremity edema were improved with the addition of furosemide. By the 7-month appointment, the patient's New York Heart Association's status had declined from class II to class III, with shortness of breath after walking 1 block. Because of her worsening cardiac status and concern that she would decompensate further during delivery, the decision was made to perform a cesarean delivery in a cardiac operating room with the immediate availability of cardiopulmonary bypass.
Multiple interdisciplinary meetings were held to discuss the delivery and anesthetic plan. The patient was admitted to the hospital on the night before scheduled cesarean delivery at 36 weeks of gestation, where an infusion of milrinone was started at 0.5 [micro]g/kg per minute (dose range, 0.375-0.75 [micro]g/kg per minute). She presented to the cardiac surgical suite, where standard American Society of Anesthesiologists monitors and oxygen via nasal cannula were applied. In addition, central venous access was placed to monitor central venous pressure (CVP), invasive arterial monitoring was started, and an epidural catheter was placed at L3-4 with loss of resistance to air technique. The patient was prehydrated with approximately 1000 mL of crystalloid to increase CVP...