Perioperative electrophysiology study in patients with tetralogy of Fallot undergoing pulmonary valve replacement will identify those at high risk of subsequent ventricular tachycardia.

Background: Ventricular tachyarrhythmias are the most common cause of death in patients with repaired tetralogy of Fallot (TOF), but predicting those at risk remains a challenge. An electrophysiology study (EPS) has been proposed to risk stratify patients with TOF.

Objective: We sought to evaluate a perioperative EPS-guided approach to risk stratify patients with TOF undergoing pulmonary valve replacement (PVR) and guide concomitant cryoablation.

Methods: A prospective cohort study of patients with TOF undergoing an EPS at the time of PVR from 2006 to 2017 was conducted at 2 centers. Patients inducible at the time of pre-PVR had undergone concomitant cryoablation in addition to PVR. A repeat post-PVR EPS was performed in those initially inducible to guide implantable cardioverter-defibrillator (ICD) implantation.

Results: Of 70 patients who underwent a pre-PVR EPS, 34 (49%) had inducible sustained ventricular tachycardia (VT): 25 monomorphic VT and 9 polymorphic VT. Among patients undergoing cryoablation, 14 (45%) had inducible VT and underwent ICD implantation. During a mean follow-up period of 6.1 ± 3.2 years, 3 patients (21%) had appropriate ICD shocks for symptomatic VT. There was an average of 2.3 shocks (range 1-4 shocks), and the mean time to first shock post-device implantation was 3.6 years (range 2.9-4.3 years). Among patients with negative pre- or post-PVR EPS results, 2 had VT requiring radiofrequency ablation and/or subsequent ICD implantation. There were no arrhythmic deaths.

Conclusion: A pre-PVR EPS identified patients with higher-risk TOF undergoing PVR. Despite empirical VT cryoablation at the time of PVR, a high percentage of patients remained inducible for VT. In this high-risk cohort, post-PVR EPS evaluation is important to identify patients at risk of VT despite cryoablation.