Pulmonary regurgitation and prolonged QRS duration of right bundle branch (RBB) block are common in repaired tetralogy of Fallot (TOF) and increase the risk of sudden death. We sought to establish an animal model to reflect both abnormalities. Twenty-one canines: group I (n ϭ 7) received a surgical right ventricular outflow tract (RVOT) transannular patch plus pulmonary valve destruction; group II (n ϭ 5) received RBB ablation and sham operation; and group III (n ϭ 9) received combined interventions. Serial electrophysiological data were obtained up to 1 y. Procedure mortality was 27.6%. At 1 y, although severe pulmonary regurgitation was documented in most dogs in groups I (71%) and III (100%), progressive RVOT dilatation was noted in group III. RBB block was present in all dogs in groups II and III. However, the increments of QRS duration, QTc, JTc, and QT dispersion progression between 1 mo and 1 y were all greatest in group III. Ventricular arrhythmia events were frequent in group III (median 3.3/mo) but uncommon in groups I and II (median 1/mo). We have created a novel animal model that adequately reflects both the hemodynamic and electrophysiological characteristics of repaired TOF patients and can be applied to examine the risk of ventricular arrhythmias. (Pediatr Res 70: 247-252, 2011) W ith the advances in cardiac interventions, most patients with tetralogy of Fallot (TOF) can survive into adulthood. Long-term follow-up, however, has revealed that these patients are at risk of ventricular arrhythmia and sudden cardiac death (1). Patients with repaired TOF commonly have complete right bundle branch block (RBBB), detected by surface electrocardiography (EKG) with prolonged QRS duration (2). This complication is related to the patch repair of the ventricular septal defect and the right ventricular outflow tract (RVOT) incision to relieve RVOT obstruction. Risk factors for ventricular arrhythmia disclosed in previous studies include hemodynamic factors such as severe pulmonary regurgitation (PR) and increased left ventricle end-diastolic pressure and electrophysiologic factors such as prolonged QRS duration (3,4). The possible mechanism includes a mechanoelectrical interaction that PR could increase right ventricular loading, which further aggravates QRS prolongation (5). Thus, the depolarization and repolarization inhomogeneity and secondary QT prolongation contribute to the development of ventricular arrhythmia. However, the interaction is complex and difficult to elucidate (6).Recently, several animal models have been proposed to simulate the hemodynamic changes in repaired TOF patients by using pulmonary valvotomy or transannular patch (7-11). Although effective in hemodynamic simulation, the importance of electrophysiological factor was seldom addressed. Thus, these models may not adequately represent the whole clinical scenario of repaired TOF. We therefore created a novel animal model in which RVOT transannular patch, PR creation, and RBBB by radiofrequency ablation would all be present to ...