Is ICD Implantation Useful in Patients with Arrhythmogenic Right-ventricular Cardiomyopathy?

摘要

The natural history of arrhythmogenic right-ventricular cardiomyopathy / dysplasia (ARVC/D) is closely related to ventricular electrical instability, which can precipitate sudden arrhythmic death, mostly in adolescents and young adults [1-4]. Heart failure is rare, and occurs later during the disease course as a result of progression of the right-ventricular disease and left-ventricular involvement [4]. ARVC/D has become an emerging indication for implantable-cardioverter defibrillator (ICD) to prevent sudden arrhythmic death in the setting of cardiomyopathy [5]. Although there is definitive clinical evidence that ICD is the most effective therapy for both primary and secondary prevention of sudden death in patients with coronary artery disease [6-8], there are very few published data on the efficacy and safety of such a therapy in patients with ARVC/D, mostly because of the relatively low prevalence of the disease in the general population and the relatively low event rate [9-11], Current indications for ICD implantation in patients with arrhythmogenic right-ventricular cardiomyopathy / dysplasia are empiric, being based widely on the experience gained by different centers using analogies with other conditions requiring antiarrhythmic therapy [12]. Since the identification of clinical findings that predict clinical outcome has remained elusive, there is a gathering tendency to implant a ICD indiscriminately once the disease has been diagnosed, regardless of risk stratification [12].