Congenital Coronary Artery–to–Pulmonary Artery Fistula with Anomalous Origin of Right Coronary Artery from Pulmonary Artery: A Case of “Double Trouble”

Congenital coronary artery fistula is a rare coronary anomaly. Its clinical significance focuses mainly on the mechanism of coronary steal phenomenon. A combination of left main (LM) coronary artery–to–main pulmonary artery (MPA) fistula with anomalous origin of right coronary artery from the pulmonary artery (ARCAPA) was encountered in a 3-month-old infant who presented with tachypnea. Evaluation with echocardiography and CT confirmed the diagnosis. The patient underwent surgical ligation of LM-to-MPA fistula with direct reimplantation of ARCAPA to aortic root.