Congenital atresia of the LMCA is an extremely rare anomaly which is often clinically complicated by silent angina, myocardial infarction, failure to thrive, or sudden cardiac death. Moreover, the atretic and ectopic origin of the LMCA associated with aortic valve anomalies is an even rarer condition. Herein, the case is described of a patient with a very rare association between the ectopic and atretic left main coronary artery (LMCA) and severe aortic valve regurgitation.
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