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Both of congenital giant RCA, RCA to PA fistula, and CLMCA-A are one of exceedingly rare vascular abnormalities. We present the case of a 34-year-old man with multiple congenital coronary anomalies above, and a successful surgical intervention was proceeded for our patient.  This is a case of a 34-year-old man presented with apical systolic murmur and exertional chest pain. Furthermore, a dilated left heart and abnormal pulmonary blood flow was observed in this patient. Coronary CTA and selective CAG showed a 15-mm diameter RCA with a fistula to pulmonary trunk and CLMCA-A. During cardiac surgery, the coronary abnormalities were confirmed and the outlet of the fistula was closed. After surgery, less chest pain, respiratory infection, and left heart failure were observed in this patient.  This case illustrates that the surgery of closing the fistula between the giant RCA and pulmonary trunk could improve the symptoms and signs for this patient. It could be predicted that simplified operation could be a promising therapy for patient with giant RCA to PA fistula and CLMCA-A. Moreover, CABG should be taken full account of when myocardial ischemia was found in the blood-supply area of LAD.

Congenital Giant Right Coronary Artery to Pulmonary Artery Fistula and Congenital Left Main Coronary Artery Atresia