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Atrioventricular reciprocating tachycardia (AVRT) is known to be the most common supraventricular tachycardias in childhood. Because AF with rapid ventricular response may degenerate to ventricular fibrillation through conduction of accessory pathways (APs), it can be potentially life-threatening in some pediatric patients with WPW syndrome. However, information about WPW syndrome children associated with AF is limited. The purpose of this study was to investigate the specific electrophysiological characteristics in pediatric patients with WPW syndrome and AF. From July 1992 to February 2002, 51 pediatric patients with manifest WPW syndrome and documented AVRT underwent electrophysiological study and radiofrequency catheter ablation. In these patients, two (4%) were found to have several spontaneous episodes of AF recognized on 12-lead standard ECG or 24-hour Holter monitoring. Eleven (22%) patients had AF induced by rapid atrial pacing during the baseline procedure of electrophysiological study. The children with manifest WPW syndrome were divided into two groups: those with AF (group 1; n = 11) consisted of seven male and four female children (mean age 15 ± 3 years, range 10–18), and those without AF (group 2; n = 40) consisted of 22 boys and 18 girls (mean age 16 ± 3 years, range 7–18). The study excluded a patient who had Ebstein's anomaly associated with moderate tricuspid regurgitation and right atrial enlargement. The onset and duration of symptoms were not significantly different between the two groups. Comparing the electrophysiological characteristics, the atrial effective refractory period (ERP) was shorter in WPW syndrome children with AF (170 ± 36 vs 190 ± 38 ms, P = 0.041). This study demonstrated that the pediatric WPW syndrome patients with AF had different electrophysiological characteristics from those without AF.