title: Atrial tachyarrhythmias and the Cox-maze procedure in congenital heart disease.
abstract: BACKGROUND
  Atrial tachyarrhythmias, particularly atrial flutter and fibrillation, are commonly associated with congenital heart disease and are a major cause of morbidity and mortality. The Cox-maze procedure, introduced by Dr. James Cox in 1987, is effective at controlling atrial fibrillation in structurally normal hearts. Though the Cox-maze procedure has been used for atrial tachyarrhythmias in patients with congenital heart disease, few studies have looked at its effectiveness.
  
  
  METHODS
  A retrospective chart review was performed on 24 patients with congenital heart disease who underwent the Cox-maze procedure at the Medical College of Wisconsin from 2004 through 2010.
  
  
  RESULTS
  Mean age at time of Cox-maze procedure for the cohort was 40.9 years (range, 14 to 66 years). The most common congenital heart diseases among the patients included tetralogy of Fallot (n = 8) and atrioventricular septal defect (n = 4). All patients had concomitant cardiac procedures with the most common being right ventricular outflow tract reconstruction (n = 10), tricuspid valve repair (n = 8), and atrial septal defect repair (n = 7). Prior to the Cox-maze procedure, arrhythmias consisted of atrial flutter or intratrial reentrant tachycardia (n = 19) and atrial fibrillation (n = 5). There were three early postoperative deaths and one late postoperative death. Follow-up was available for 19 of 21 (90%) survivors with a mean length to follow-up from Cox-maze procedure of 2.8 years (range, 0.14-5.7 years). At last follow-up, 14 (74%) of the survivors remained arrhythmia-free.
  
  
  CONCLUSIONS
  In patients with congenital heart disease and atrial tachyarrhythmias, the majority were rendered arrhythmia-free by the Cox-maze procedure.
authors.affiliation: Department of Pediatrics and Internal Medicine, Medical College of Wisconsin, Milwaukee, Wis, USA.
authors.forename: Sherrill D
authors.initials: SD
authors.lastname: Gutierrez
authors.forename: Michael G
authors.initials: MG
authors.lastname: Earing
authors.forename: Anoop K
authors.initials: AK
authors.lastname: Singh
authors.forename: James S
authors.initials: JS
authors.lastname: Tweddell
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Bartz
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001281:Atrial Fibrillation / Q000150:complications / Q000401:mortality / Q000601:surgery*; D001282:Atrial Flutter / Q000150:complications / Q000401:mortality / Q000601:surgery*; D017115:Catheter Ablation; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000150:complications / Q000401:mortality / Q000601:surgery*; D006343:Heart Septal Defects / Q000150:complications / Q000401:mortality / Q000601:surgery; D006344:Heart Septal Defects, Atrial / Q000150:complications / Q000401:mortality / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000401:mortality; D012189:Retrospective Studies; D013771:Tetralogy of Fallot / Q000150:complications / Q000401:mortality / Q000601:surgery; D055815:Young Adult
keywords: Atrial Fibrillation; Atrial Flutter; Congenital Heart Disease; Maze