title: The Pediatric Radiofrequency Ablation Registry's experience with Ebstein's anomaly. Pediatric Electrophysiology Society.
abstract: BACKGROUND
  Abnormal anatomy and complex electrophysiology in patients with Ebstein's anomaly of the tricuspid valve may confound attempts at radiofrequency ablation (RFA).
  
  
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: The Children's Heart Center, Department of Epidemiology, Rollins School of Public Health, Emory University School of Medicine, Atlanta, Georgia 30322, USA.
authors.forename: J D
authors.initials: JD
authors.lastname: Reich
authors.forename: D
authors.initials: D
authors.lastname: Auld
authors.forename: E
authors.initials: E
authors.lastname: Hulse
authors.forename: K
authors.initials: K
authors.lastname: Sullivan
authors.forename: R
authors.initials: R
authors.lastname: Campbell
mesh_terms: D000293:Adolescent; D000328:Adult; D017115:Catheter Ablation / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D004437:Ebstein Anomaly / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery*; D004594:Electrophysiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D012042:Registries; D012189:Retrospective Studies; D013617:Tachycardia, Supraventricular / Q000209:etiology / Q000601:surgery*; D016896:Treatment Outcome; D014481:United States