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

Abstract

Objective-To study the outcome of patients with arrhythmogenic right ventricular 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 with ventricular tachycardia and haemodynamic collapse (n = 6) or ventricular 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 alive. Six patients received a median of 19 (range 2-306) appropriate ICD interventions for events detected in the ventricular tachycardia window; four received a median of 2 (range 1-19) appropriate ICD interventions for events detected in the ventricular fibrillation window. Inappropriate interventions were seen for sinus tachycardia (18 episodes in three patients), atrial fibrillation (three episodes in one patient), and for non-sustained polymorphic ventricular tachycardia (one episode in one patient). Conclusions-Patients with arrhythmogenic right ventricular dysplasia and malignant ventricular arrhythmias have a high recurrence rate requiring appropriate ICD interventions, but they also often have inappropriate interventions. Programming the device is diYcult because this population develops supraventricular and ventricular tachyarrhythmias with similar rates. (Heart 2001;85:53-56)

Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia: Risk Stratification and Therapy

“…The results of published trials strongly suggest an improvement in long-term prognosis by ICD therapy in high-risk patients with ARVC/D (Tab 2) (17)(18)(19)(20)23). The estimated benefit of ICD implantation in preventing potentially fatal events (i.e.…”

“…In summary, the available data suggest that young age (<35 years old), prior cardiac arrest, fast and poorly-tolerated VT with different morphologies, syncope, severe right ventricular dysfunction, heart failure with left ventricular involvement, familial occurrence of juvenile sudden death, are the major determinants in predicting sudden death and worse outcome (4)(5)(6)(7)(8)(16)(17)(18)(19)(20)(21)(22)(23)(24)31,32). In patients with ARVC/D vigorous or competitive sport activities should be restricted to decrease the rate of structural and/or functional progression of the disease as well as the risk of arrhythmic death.…”

“…Several studies regarding ICD implantation in ARVC/D are published (16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27), and the main data from some of them are summarized in Table 2 and Figure 1. There is a predominance of male gender with a mean age of nearly 40 years.…”

“…(16,17,(19)(20)(21)(22)(23). Algorithm for the antiarrhythmic management of ARVC/D Table 2 Studies on ICD Therapy in ARVC/D (modified from 24) NR= not reported; Prev.= prevention; Pts=patients; SC= single-center study; MC=multicenter study; Ther.=therapy; FU=follow-up; Mo=months.…”

“…The role of pharmacological therapy in controlling VA and preventing sudden death has been evaluated in single-center studies (9), and the results of catheter ablation have been described in small series of patients (10-15). The efficacy and safety of implantable cardioverter/ defibrillator (ICD) have also been reported in small, single-center studies (16)(17)(18)(19) and recently in larger single and multicenter studies (20)(21)(22)(23)(24)(25)(26)(27). The main questions regarding the risk stratification and the therapeutic strategy in ARVC/D are: 1) differential diagnosis with idiopathic VT (right ventricular outflow tract VT) in an apparently normal heart.…”