Introduction: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare Cardiovascular Disease presented with an incidence of 1:300000 live births. Case Presentation: In this manuscript, four cases of ALCAPA in infancy were described. Two infants were presented with respiratory distress and two with heart a murmur. Their coronary artery was derived from the pulmonary artery. Two of them died, one after surgical procedure and one before surgical intervention. Of remained cases, one of them had a good surgical outcome and another was missed in follow up. A brief review was done on case reports of ALCAPA in children. Eighteen manuscripts were found including 201 pediatric cases. Conclusions: The vast majority of affected children will die within the first year of life if ALCAPA is not diagnosed and treated correctly.
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