Single coronary artery and coronary artery-pulmonary artery fistula in a variation of the Shone complex.

We aim to share the diagnostic methods and relevant findings of a rare case involving a 1-year-old girl with a variation of Shone syndrome, presenting with the coexistence of a single coronary artery and a fistula between the coronary artery and the right pulmonary artery. The patient had previously undergone aortic arch reconstruction surgery and during the preoperative evaluation, cardiac computed tomography angiography (CCTA) revealed the additional presence of a single coronary artery originating from the right, which fistulized into the right pulmonary artery distally. This finding was further confirmed by conventional angiography. This case report highlights the importance of CCTA in identifying and directing the treatment of additional anomalies that may accompany rare congenital abnormalities.