A 22-year-old female with cyanotic congenital heart disease was found to have veno-arterial shunting via a large secundum atrial septal defect despite normal pulmonary arterial and right ventricular pressures. Persistence of the Eustachian valve of the inferior vena cava was suspected preoperatively and confirmed at operation. This valve caused a large right-to-left shunt by favoring the flow of inferior vena caval blood into the left atrium via the defect. Surgical excision of the abnormal valve and closure of the atrial septal defect resulted in complete physiological and clinical cure.
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