A one day old baby who presented with cyanosis was later shown to have an isolated persistent left superior vena cava draining into the left atrium with hypoplastic right superior vena cava. This extremely rare anomaly was diagnosed by cardiac catheterisation at one week. Surgical correction at two weeks, by anastomosis of the innominate vein to the right atrium and by ligation of the left superior vena cava at its junction with the left atrium, was successful. This patient is the youngest case in which successful surgical correction of this isolated anomaly has been reported.
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