title: Supraventricular tachyarrhythmias in Ebstein anomaly: management and outcome.
abstract: OBJECTIVE
  This study was undertaken to review the role of electrophysiology testing and to determine the early and late results of medical and surgical management of supraventricular tachyarrhythmias in Ebstein anomaly.
  
  
  METHODS
  We reviewed 130 patients between 1990 and 2001 with Ebstein anomaly and history of tachyarrhythmia with a median age of 25 years (mean age 27.5 years); 106 underwent electrophysiologic testing and 24 had documented atrial flutter or fibrillation. We excluded 21 patients: negative results of testing (n = 18), ventricular tachycardia (n = 2), and junctional tachycardia (n = 1). The remaining 109 patients had more than one mechanism: accessory pathway-mediated tachycardia (n = 49), atrioventricular nodal reentrant tachycardia (n = 10), and atrial flutter/fibrillation (n = 70).
  
  
  RESULTS
  Eighty-three patients underwent at least one arrhythmia procedure combined with surgery for Ebstein anomaly. Early mortality was 4.8%. Forty-one patients underwent surgical ablation of an accessory pathway without recurrent accessory pathway-mediated tachycardia at a mean follow-up of 48 months. Seven patients underwent surgical perinodal cryoablation for atrioventricular nodal reentrant tachycardia without recurrence at a mean follow-up of 57 months. Forty-eight patients underwent surgical intervention for atrial flutter or fibrillation (right-sided maze procedure, n = 38, and cryoablation of the atrial isthmus, n = 10). Freedom from recurrent atrial flutter or fibrillation was 75% at a mean follow-up of 34 months.
  
  
  CONCLUSIONS
  Concomitant arrhythmia procedures can be performed without increase in early mortality and should be added to Ebstein repairs for all patients who have supraventricular tachyarrhythmias. Surgical procedures for accessory pathway-mediated tachycardia and atrioventricular nodal reentrant tachycardia give excellent (100%) freedom from recurrence of those arrhythmias. Surgical intervention for atrial flutter/fibrillation yields freedom from late recurrence in 75% of cases.
authors.affiliation: Department of Pediatric and Adolescent Medicine, Division of Pediatric Cardiology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
authors.forename: Anant
authors.initials: A
authors.lastname: Khositseth
authors.forename: Gordon K
authors.initials: GK
authors.lastname: Danielson
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Dearani
authors.forename: Thomas M
authors.initials: TM
authors.lastname: Munger
authors.forename: Coburn J
authors.initials: CJ
authors.lastname: Porter
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D006348:Cardiac Surgical Procedures; D017115:Catheter Ablation; D002648:Child; D002675:Child, Preschool; D004437:Ebstein Anomaly / Q000150:complications*; D022062:Electrophysiologic Techniques, Cardiac; D005260:Female; D006329:Heart Conduction System / Q000002:abnormalities / Q000601:surgery; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D013611:Tachycardia, Atrioventricular Nodal Reentry / Q000601:surgery; D013617:Tachycardia, Supraventricular / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*