Extracardiac conduit with a limited maze procedure for the failing Fontan with atrial tachycardias

BACKGROUND: Atrial arrhythmias are a frequent late complication of Fontan procedures. Conversion to an extracardiac conduit combined with reducing right atrial size should improve hemodynamics and reduce the development of tachyarrhythmias. More effective control may be achieved by interrupting atrial arrhythmia circuits and atrial pacing. METHODS: Between May 1997 and October 2001, 6 patients underwent a revision of their intracardiac Fontan anastomosis. The conversion included an extracardiac conduit insertion, limited right atrial maze procedure, and pacemaker placement. Ages ranged from 14 to 34 years (mean, 22.8 years) at an average of 14.6 +/- 4.4 years after their original Fontan procedure. All of the patients had medically uncontrollable atrial tachyarrhythmias with markedly reduced exercise tolerance. RESULTS: All of the patients survived with an average hospital stay of 16.7 days. Exercise tolerance has improved in all 6 patients, and atrial tachycardias have either decreased (n = 2) or disappeared (n = 4). Two patients are taking antiarrhythmic medications other than digoxin. Follow-up is a mean of 28.5 months. CONCLUSIONS: Limited right atrial maze procedure, atrial size reduction, and pacemaker implantations are worthwhile additions to simple conversion to an extracardiac conduit in the failing Fontan.