Long term results of cardioverter-defibrillator implantation in patients with right ventricular dysplasia and malignant ventricular tachyarrhythmias

Objective-To study the outcome of patients with arrhythmogenic right ventri cular dysplasia treated with an implantable cardioverter-defibrillator (ICD ) for ventricular tachyarrhythmias complicated by haemodynamic collapse. Design-Observational study. Setting-University hospital. Patients-Nine consecutive patients (eight male, one female; mean (SD) age, 36 (18) years) with arrhythmogenic right ventricular dysplasia presenting w ith ventricular tachycardia and haemodynamic collapse (n = 6) or ventricula r fibrillation (n = 3), treated with an ICD. Main outcome measures-Survival; numbers of and reasons for appropriate and inappropriate ICD interventions. Results-After a mean (SD) follow up of 32 (24) months, all patients were al ive. Six patients received a median of 19 (range 2-306) appropriate ICD int erventions for events detected in the ventricular tachycardia window; four received a median of 2 (range 1-19) appropriate ICD interventions for event s detected in the ventricular fibrillation window. Inappropriate interventi ons were seen for sinus tachycardia (18 episodes in three patients), atrial fibrillation (three episodes in one patient), and for non-sustained polymo rphic ventricular tachycardia tone episode in one patient). Conclusions-Patients with arrhythmogenic right ventricular dysplasia and ma lignant ventricular arrhythmias have a high recurrence rate requiring appro priate ICD interventions, but they also often have inappropriate interventi ons. Programming the device is difficult because this population develops s upraventricular and ventricular tachyarrhythmias with similar rates.