Coronary artery fistula connecting the left main coronary artery with the superior vena cava

Background: Coronary artery fistulas are rare congenital coronary artery abnormalities, with direct communication between a coronary artery and a cardiac chamber, great vessel or other structure. Description of case: We report a case of a large coronary artery fistula connecting the left main coronary artery with the superior vena cava in a 70-year-old patient undergoing diagnostic coronary angiography for a non-ST-segment elevation myocardial infarction. The patient rejected closure of the fistula and remains asymptomatic on follow-up. Conclusion: Angiographic recognition of coronary artery fistulas is important for the appropriate diagnosis and management of patients.