Anomalous Left Main Coronary Artery Origin from Right Pulmonary Artery with Preserved Left Ventricular Systolic Function and Myxomatous Degeneration of Mitral Valve

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome is a rare congenital anomaly. It affects 1 of every 300,000 live births. Up to 90% of patients with ALCAPA syndrome die within the 1st year of life due to “coronary steal” phenomenon. We present a case of 8-year-old girl with anomalous origin of left main coronary artery from right pulmonary artery with preserved left ventricular systolic function, associated with myxomatous degeneration of mitral valve and severe MR.