Therapeutic management in Sicilian patients with definite arrhythmogenic right ventricular dysplasia/cardiomyopathy and focus on the role of implantable cardioverter-defibrillator therapy

Abstract

Arrhythmogenic Right Ventrticular Dysplasia/Cardiomyopathy (ARVD/C) is an inherited cardiomyopathy characterized by right ventricular myocyte loss with fibrofatty replacement, a high risk of ventricular arrhythmias (VA) and sudden cardiac death (SCD) [1]. Prevention of SCD represents the most important management strategy and the achievement of this target can be reached by different therapeutic strategies including implantable cardioverter-defibrillator (ICD) implantation, pharmacologic therapy, catheter ablation of ventricular tachycardia (VT) and cardiac transplantation [2,3]. The aim of this study is to examine the outcome of the different therapies adopted in a group of affected patients, focusing on the role and predictors of ICD therapy.We conduced a multicenter study evaluating 28 patients (18 male; age 42 ± 14 years) with definite ARVD/C. Diagnosis of ARVD/C was based upon the 2010 revised Task Force Criteria [4] and only patients with definite diagnosis entered the study to enhance diagnostic specificity. Management therapy was established according to the clinical features and risk stratification of each patient. All patients were followed up at biannual and yearly intervals and data included invasive and noninvasive investigation, and device interrogation. The estimate of the potential survival benefit of ICD was limited to appropriate ICD shock therapies for episode of VF/VFL, since not any arrhythmic events necessarily correspond to the true arrhythmic risk of death. We studied our patients over a mean follow-up of 6.0 ± 4.4 years.Antiarrhythmic drugs were used in 26 patients (93%), and in 13 patients (50%) this therapy was associated with ICD implantation [Sotalol in 11 patients (42%) such as amiodarone; β-blockers in 4 patient (15%)]. During the follow-up 8 patients (61%), that initially received only antiarrhythmic drugs, had an ICD implantation cause of the lack of arrhythmic control. Radiofrequency catheter ablation was performed in 6 patients (21%) and in no cases its efficacy was observed because of the recurrence of VT that required ICD implantation in whole cases. Cardiac transplantation was performed as a final therapeutic option due to refractory congestive heart failure in 3 patients (10%).At the time of the diagnosis 15 patients (54%) received an ICD implantation based to the estimated risk of SCD, according with the last guidelines [5] for management of patients with VA. During the follow-up 8 patients (61%) received an ICD because of the relapse of arrhythmic events although they were using antiarrhythmic drugs or underwent catheter ablation. Twenty patients (86%) had received appropriate ICD therapy. An appropriate ICD shock intervention for ventricular fibrillation (VF)/ventricular flutter (VFL) was seen in 12 patients (52%). Compared with the 100% actual survival rate, VF/VFL-free survival rate was 96%, 94% and 51% respectively at 1, 5 and 10 years of follow-up (logrank p b 0.0001) (Fig. 1). The estimated mortality reduction at 1, 5, 10 years of follow-up was 4%, 6% and 49% and th...

Usefulness of antitachycardia pacing in arrhythmogenic right ventricular dysplasia/cardiomyopathy

“…It usually manifests clinically with sustained monomorphic ventricular tachycardia (VT) with left bundle morphology in young individuals during exercise and can cause sudden death (SD), so the most important decision is whether or not to implant an implantable cardioverter-defibrillator (ICD) [3][4][5]. There is general agreement that ICD therapy is indicated in patients who survived an episode of ventricular fibrillation (VF) or sustained VT because of their high incidence of arrhythmia recurrences, which do not have an impact for their survival.…”

Treatment decision making in patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy: State of the art

“…Several studies of ARVD/C probands who received an ICD showed appropriate interventions during follow-up in more than 50% of patients and approximately 40% were considered life-saving based on the presence of rapid VT/VF [8,9]. We recently reported the results of a study that investigated the therapeutic management in a group of ARVD/C patients [10]. Twenty-three patients received an ICD, and over a mean follow-up of 6.0 ± 4.4 years, 20 patients (86%) had received appropriate ICD therapy.…”