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The clinical detail, echocardiographic features, angiographic evaluation, and surgical repair of double-outlet left atrium (LA) in a 2-year-old boy are discussed. In a solitus position and normally connected systemic and pulmonary venous drainage setting, the only outlet of the right atrium was a secundum atrial septal defect. The LA thus received the entire venous return and then drained into both ventricles through a common atrioventricular valve.