The Pediatric Radiofrequency Ablation Registry's experience with Ebstein's anomaly. Pediatric Electrophysiology Society.

INTRODUCTION: Abnormal anatomy and complex electrophysiology in patients with Ebstein's anomaly of the tricuspid valve may confound attempts at radiofrequency ablation (RFA).

METHODS AND RESULTS: Data for 65 pediatric Ebstein's patients (9.8+/-5.4 years, 4 months to 20 years; 39+/-25 kg, 5.1 to 108 kg) were obtained from the Pediatric Radiofrequency Ablation Registry. The degree of tricuspid regurgitation (DOTR) and the degree of Ebstein's anomaly were assessed with echocardiography/Doppler. Leading indications were drug refractoriness (24 [37%] of 65 patients) and life-threatening arrhythmia (14 [22%] of 65 patients). For the 65 patients, 82 typical (nondecremental) accessory pathways (APs) (62% right free wall, 34% right septal, and 4% left sided), 17 other supraventricular tachycardias (1 ectopic atrial, 7 AV reentry, 5 Mahaim, and 4 intra-atrial reentry tachycardias), and 1 ventricular mechanism were mapped. Thirty-four (52%) of 65 patients had a single AP (21 right free wall, 10 septal, and 3 left); 19 (29%) of 65 patients multiple APs; 6 (9%) of 65 patients a single AP plus a non-AP mechanism; and 6 (9%) of 65 patients non-AP mechanism(s) only. RFA acute success rates and recurrence rates for right free wall, right septal, and other mechanisms were 79%/32%, 89%/29%, and 75%/27%. Mild DOTR and a body surface area (BSA) < or = 1.7 m2 independently predicted a better acute success rate. BSA < or = 1.7 m2 also predicted long-term success.

CONCLUSION: In this patient subset, life-threatening arrhythmias and multiple electrophysiologic mechanisms are commonly encountered during RFA. Mild DOTR and a BSA < or = 1.7 m2 predict a higher acute success rate. While acute success rates are relatively high, recurrence is frequent.