2021 Volume 25 Issue 1 Pages 43-47
A 43-day-old girl had been diagnosed with scimitar syndrome with right pulmonary hypoplasia and scimitar veins, atrial septal defect, and aortopulmonary collateral artery during the neonatal period. Pulmonary hypertension due to a high pulmonary blood flow was difficult to control, and after coil embolization of the aortopulmonary collateral artery, atrial septal defect closure and ductus arteriosus ligation were performed. Intraoperatively, the patient's blood pressure was markedly reduced after displacement of the heart, making cannulation into the ascending and descending vena cava difficult. Pulmonary artery cannulation was performed instead, and cardiopulmonary bypass was done to complete the operation. In cases of scimitar syndrome, which requires treatment intervention in early infancy, pulmonary hypoplasia and multiple left-to-right shunts affect the right ventricular volume and pressure overload. Unexpected intraoperative circulatory collapse and postoperative pulmonary hypertension crises may occur.
