Abstract
Congenital atresia of the left main coronary artery (LMCA) is a rare anomaly in which the LMCA does not receive antegrade flow from any vessel and instead receives exclusively retrograde flow from right coronary artery (RCA) collaterals. Its estimated incidence is 0.04%.1 Clinical presentation is varied and can manifest as heart failure and sudden cardiac death in infancy and childhood or as angina in adulthood.2 We present two pediatric cases with different clinical presentation, subtle initial diagnostic findings, and surgical management challenges.
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