Anesthetic management for Norwood procedure in a neonate with Pentalogy of Cantrell

Abstract

　Pentalogy of Cantrell is a rare congenital syndrome characterized by 5 major malformations: defects involving the abdominal wall, lower sternum, pericardium, and diaphragm; and various congenital cardiovascular anomalies. We herein present the perioperative management of a neonate with Pentalogy of Cantrell, including coarctation complex, subvalvular aortic stenosis, and ectopia cordis. Because of uncontrollable pulmonary overcirculation, the Norwood procedure with a right ventricle-pulmonary artery conduit and internalization of the ectopic heart was performed on the second day after birth. The sternal defect was covered with skin after the completion of the surgery, and subsequently, we had to achieve balanced respiratory and circulatory status by adjusting the ventilator settings. Due to the lack of a pericardial cavity owing to Pentalogy of Cantrell, close monitoring of hemodynamic and ventilatory parameters is essential to control the cardiac and respiratory functions in univentricular parallel circulation.