Left Main Coronary Artery Atresia

BACKGROUND: Angina is commonly associated with atherosclerotic epicardial coronary artery disease, but it could be the presenting symptom of a coronary structural anomaly or even a noncoronary cardiac structural pathology. CASE SUMMARY: A 40-year-old man with obesity, hypertension, and hyperlipidemia presented with exertional chest pain and dyspnea. Coronary computed tomographic angiography showed no coronary atherosclerosis. However, there was an absence of a direct left main coronary artery connection to the aortic root, and retrograde filling of the left coronary system from right coronary collateral vessels. Stress echocardiography demonstrated no wall motion abnormality or ventricular arrhythmia. Medical management, including beta-blockade, aggressive risk factor modification, and surveillance, was pursued through shared decision-making. DISCUSSION: Left main coronary artery atresia is an exceedingly rare congenital anomaly that may present as exertional angina mimicking atherosclerotic coronary artery disease. Coronary computed tomographic angiography provides precise anatomical information for the diagnosis, and functional testing helps guide individualized management.