Tetralogy of Fallot and pulmonary atresia/ventricular septal defect

Surgical repair of tetralogy of Fallot is associated with excellent results. Long-term complications include arrhythmias, sudden death, and right ventricular aneurysm formation. Reoperation is necessary in approximately 5% of patients for residual ventricular septal defect, pulmonary regurgitation, or pulmonary stenosis. Pulmonary atresia/ventricular septal defect is a more complex situation, and the anatomy needs careful delineation by cardiac catheterization prior to any surgical intervention. Palliation with a shunt or first-stage repair may be effective, or radical repair may be considered in those with adequate pulmonary distribution.