Anesthetic management for cesarean section in a patient with uncorrected double-outlet right ventricle

BACKGROUND: We describe the anesthetic management for cesarean section in a pregnant woman with uncorrected double-outlet right ventricle. The anesthetic method, treatment of complications and lessons are discussed. CASE PRESENTATION: A 28-year-old woman visited our emergency room for progressive dyspnea and recurrent hemoptysis at 30 weeks' gestation. Her New York Heart Association functional class was III-IV. Echocardiography indicated that she had congenital heart disease of double-outlet right ventricle. She hadn't received any treatment. The obstetrician decided to terminate the pregnancy by cesarean section. We chose epidural anesthesia and pumped phenylephrine at the same time to minimize hemodynamic fluctuation. Just in the process of changing position to supine position with uterus displaced to the left, the patient coughed badly and complained about dyspnea. At the same time, the oxygen saturation decreased quickly. The symptoms were ameliorated soon by treating as heart failure. But the symptoms reappeared after oxytocin administration. At the end of surgery, the baby was sent to the Neonatal Intensive Care Unit for premature birth. The mother recovered successfully and discharged 7 days later. DISCUSSION AND EVALUATION: Double-outlet right ventricle is a seldom disease and pregnancy with uncorrected double-outlet right ventricle is rare. In this case, the patient belonged to the type of ventricular septal defect characterized by subaortic ventricular septal defect without pulmonary stenosis. Most of the aorta arises from the right ventricle, the volume of venous blood was injected from the right ventricle into the aorta, which decided the oxygen saturations. Compared to general anesthesia, epidural anesthesia reduces venous return and alleviates the cardiac burden. So, we pumped phenylephrine along with epidural anesthesia in case of critical cyanosis following significant blood pressure decrease. In the process of anesthesia, dyspnea, cough and cyanosis attacked the patient for two times. The most probable reason was heart failure which induced by the sudden increasing of returned blood. As a result of severe cough, pulmonary artery pressure increased rapidly. Then a great amount of venous blood was injected into the aorta and cyanosis became more serious as well as oxygen saturation declined quickly. CONCLUSION: Epidural anesthesia with continuous phenylephrine infusion is a preferable choice for parturient women with uncorrected double-outlet right ventricle for cesarean section. It is not the optimal choice for this type of patients to lie on the left. The dose of oxytocin should be reduced to avoid potential cardiovascular complications.