ABSTRACT
Introduction@#Single coronary artery and coronary artery fistulas are unusual findings either alone or in combination. They may be incidental findings or can present with myocardial ischemia.@*Case Presentation@#A 46-year-old male had chest pain accompanied by heart failure symptoms. He sought consult in our institution where further workups revealed that he suffered an acute coronary syndrome. He underwent diagnostic coronary angiogram which showed significant coronary artery disease. There was also a single coronary artery with a coronary artery fistula. Patient was appraised regarding surgical revascularization and repair but did not consent. He was stabilized and improved on optimal anti ischemic therapy.@*Discussion@#Electrocardiogram revealed ST elevation myocardial infarction of the anterior wall. Two dimensional echocardiogram showed depressed systolic function with an ejection fraction of 48% and multisegmental wall motion abnormalities. Coronary angiogram revealed a 70-80% stenosis of the mid portion of the left anterior descending artery. There is a fistulous vessel draining to the main pulmonary artery. The left circumflex is super dominant with an 80-90% stenosis at its termination and supplying the right coronary circulation. The right coronary artery was absent.@*Conclusion@#Coronary artery fistula in combination with a congenitally absent coronary artery is extremely rare. The presence of these abnormalities alone or in combination can exacerbate ischemia, complicate pre existing coronary artery disease and may cause ischemic cardiomyopathy. This case emphasizes the importance of integration of clinical as well as imaging modalities to diagnose this uncommon abnormality and in guiding clinicians for definitive management.