Atrial tachyarrhythmias and the Cox-maze procedure in congenital heart disease.

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.