Clinical ventricular tachycardia and surgical epicardial ICD implantation in a patient with a Fontan operation for double-inlet left ventricle.

The Fontan operation is the primary surgical technique used for palliation of patients with single-ventricle physiology. Arrhythmias are frequently observed and associated with morbidity and mortality in Fontan patients. The frequency of arrhythmias after the Fontan procedure increases over time and it was reported to reach 50% in a 20-year follow up. Atrial tachyarrhythmias, especially atrial tachycardia and sinus bradycardia, are most frequently observed in these patients. Ventricular arrhythmias are rarely observed. Generally, medical therapy, catheter ablation, pacemaker or implantable cardioverter defibrillator (ICD) implantation are options in the treatment of these arrhythmias. It may be difficult to implant either a pacemaker or an ICD in patients on whom the Fontan procedure has been performed. In conditions where access to the right ventricle is from the venous system, it is anatomically impossible. Where there is no functional right ventricle, device implantation can be performed with alternative methods other than the conventional transvenous approach. In this report, we discuss a middle-aged woman with a Fontan operation performed 14 years earlier, who presented with ventricular tachycardia (VT) and in whom an epicardial ICD was implanted. The literature on this issue is also reviewed.