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Atrial tachyarrhythmias and the Cox-maze procedure in congenital heart disease. Congenit Heart Dis 2013 Sep-Oct;8(5):434-9



Pubmed ID




Scopus ID

2-s2.0-84884759864   18 Citations


INTRODUCTION: 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.

Author List

Gutierrez SD, Earing MG, Singh AK, Tweddell JS, Bartz PJ


Peter J. Bartz MD Professor in the Pediatrics department at Medical College of Wisconsin
Anoop K. Singh MD Associate Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Atrial Fibrillation
Atrial Flutter
Catheter Ablation
Follow-Up Studies
Fontan Procedure
Heart Defects, Congenital
Heart Septal Defects
Heart Septal Defects, Atrial
Middle Aged
Postoperative Complications
Retrospective Studies
Tetralogy of Fallot
Young Adult
jenkins-FCD Prod-482 91ad8a360b6da540234915ea01ff80e38bfdb40a