A combination of tetralogy of Fallot( TOF) and total anomalous pulmonary venous return(TAPVR) is rare and results in chronic volume and pressure load of the right side of the heart and underfilling of the left heart. We report a successful 2-staged surgical correction of TOF associated with TAPVR and atrial septal defect. The patient was unsuitable for total primary intracardiac correction because the volume of the left ventricle was considered to be small. First, repair of anomalous pulmonary venous return and palliative right ventricle outflow tract reconstruction were simultaneously performed in 2 months of birth. One year after 1st operation, cardiac catheterization revealed that normalization of left ventricle volume, so 2nd operation was planned. Total correction of ventricular septal defect and right ventricle outflow reconstruction was performed and the patient was discharged on the 21st postoperative day with good hemodynamic status.
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