BACKGROUND: Arrhythmia surgery has favorably impacted the clinical course of debilitating atrial and ventricular arrhythmias in patients with and without congenital heart disease. This study reviews arrhythmia mechanisms and documents long-term outcome of patients undergoing arrhythmia operations alone or associated with congenital heart repairs. The analysis excludes Fontan conversion patients. METHODS: Between 1987 and 2007, arrhythmia operations were done in 11 patients without associated congenital heart disease and in 89 along with congenital heart repairs. Mean age was 15.9 +/- 12.5 years (range, 7 days-48 years); 7 were infants (mean age, 23 +/- 16 days). Resternotomy was performed in 65 (65%). Two functional ventricles were present in 67 patients; 33 had 1 functional ventricle. Arrhythmias included macro-reentrant atrial tachycardia in 45, atrial fibrillation in 11, accessory connections in 19, atrioventricular nodal reentry tachycardia in 6, focal atrial tachycardia in 6, and ventricular tachycardia in 13. RESULTS: Operative mortality was 3 (3.0%) due to advanced associated congenital heart disease. There were 4 late deaths (4.0%) and 2 late cardiac transplants (2.0%). Freedom from arrhythmia recurrence at 1 and 10 years was 94% and 85% for atrial arrhythmias, and 85% and 68% for ventricular arrhythmias, respectively. CONCLUSIONS: Successful surgical therapy for atrial arrhythmias can be performed safely with a high freedom from recurrence rate in patients with and without associated congenital heart disease. Surgical ablation for ventricular arrhythmias is less predictive. Complexity of the underlying congenital heart disease and hemodynamic status may contribute to potential arrhythmia recurrence or new onset arrhythmia manifestation.