General anesthesia for cesarean section in a parturient with a single ventricle and pulmonary atresia

The successful management of a cesarean section in a parturient with a single ventricle and pulmonary atresia using general anesthesia is discussed. After cyanosis at birth, the patient underwent cardiac catheterization, which showed an apparent severe tetralogy of Fallot, atresia of the main pulmonary artery (PA), and a large patent ductus arteriosus. When she was 7 months of age, a Blalock-Taussig shunt (right subclavian artery to right PA) was done. She remained stable until age 11, when cyanosis increased and exercise tolerance decreased. Recatheterization more clearly defined the lesion: closed shunt, pulmonary valvular atresia, severe ductal stenosis, reduced pulmonary flow, double-outlet right ventricle, and severe hypoplasia of the left atrium, mitral valve, and left ventricle. A Potts shunt (left descending aorta to left PA) was done. Compliance with therapy was poor and follow-up difficult. Exercise tolerance was poor, but the patient remained otherwise stable. At 28 weeks' gestation, this 23-year-old parturient presented with severe congestive heart failure (CHF). After initial therapy with oxygen, bed rest, digoxin, and diuretics, she improved and remained stable for a month. At that time (32 weeks' gestation), CHF worsened. Because the cervix was unfavorable for a vaginal delivery, a cesarean section was planned. The patient was then taken to the operating room electively, and an opioid-based general anesthetic was administered. Both mother and infant did well. This case is presented because the physiology of the patient's lesion and her unusual social history presented challenges for her anesthetic management.