Surgical Treatment of a Rare Case of Uhl Anomaly, Tricuspid Atresia, Absent Pulmonary Valve, Hypoplastic Right Ventricle, and Right Ventricular Coronary Artery Fistula.

We report the case of a four-month-old child with complex congenital heart disease, including tricuspid atresia, absent pulmonary valve, hypoplastic right ventricle, right ventricular coronary artery fistula, and Uhl anomaly, and to describe the challenges and management decisions involved. A Blalock-Taussig-Thomas (BTT) shunt was initially planned. However, severe ST-segment depression occurred upon oral feeding, indicating coronary insufficiency. Cardiac catheterization revealed a single coronary artery with a right ventricular coronary artery fistula, which increased the risk of myocardial ischemia with the BTT shunt. Consequently, a bidirectional Glenn (BDG) procedure with left pulmonary artery reconstruction was performed after maintaining ductal patency with prostaglandin for four months. The patient had a favorable postoperative course without ischemic events. In patients with complex congenital heart defects and coronary anomalies, the presence of a right ventricular coronary artery fistula may increase the risk of ischemia with a BTT shunt. A BDG procedure can offer a safer alternative, leading to a positive clinical outcome and avoiding ischemic complications.