A girl who was diagnosed at 8 months old with anomalous left coronary artery from the pulmonary artery and was subsequently lost to follow-up, presented at the age of 11 years with congestive heart failure and severe mitral regurgitation with supra-systemic pulmonary artery pressure. With progressive mitral regurgitation, coronary steal is reduced by the rising pulmonary artery pressure, and patients present late with severe valvular cardiomyopathy.
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