Staged Single Ventricle Palliation with Pulmonary Artery Rehabilitation for Unguarded Tricuspid Orifice and Hypoplastic Left Pulmonary Artery

The patient was diagnosed with unguarded tricuspid orifice (UGTO), functional pulmonary atresia, and left pulmonary artery hypoplasia. In view of the severe right ventricular dysfunction, the staged single ventricular palliation procedure was selected. The surgical procedure on Starnes operation and left pulmonary artery augmentation was performed one month after birth. As left pulmonary artery stenosis was diagnosed, secondly, the bidirectional cavopulmonary shunt with additional systemic to pulmonary shunt and intrapulmonary patch septation and left pulmonary artery augmentation with in-situ pericardium were performed. Despite the necessity for additional balloon dilation and surgical blunt dilation, the total cavopulmonary connection operation was ultimately performed, resulting in the successful implementation of staged single-ventricle palliation in conjunction with left pulmonary artery rehabilitation.