Total anomalous pulmonary venous return with an intact atrial septum is an uncommon lesion. With associated mitral stenosis each lung has a different pulmonary flow, individual vascular resistance, and function. This represents the first reported case of this anomaly treated by total correction and mitral valve replacement. Pulmonary flow reverted to normal distribution and heart size decreased, with a reversion of vascular changes on the chest radiograph one year after operation.
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