Abstract
IntroductionArrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetically transmitted disease prone to ventricular arrhythmias. We therefore investigated the clinical, echocardiographical and electrophysiological predictors of appropriate implantable cardioverter defibrillator (ICD) therapy in patients with ARVC.MethodsA retrospective analysis was performed in 26 patients (median age of 40 years at diagnosis, 21 males and 5 females) with ARVC who underwent ICD implantation.ResultsOver a median (range) follow-up period of 10 (2.7, 37) years, appropriate ICD therapy for ventricular arrhythmias was documented in 12 (46%) out of 26 patients. In all patients with appropriate ICD therapy the ICD was originally inserted for secondary prevention. Median time from ICD implantation to ICD therapy was 9 months (range 3.6, 54 months). History of heart failure was a significant predictor of appropriate ICD therapy (p = 0.033). Left ventricular disease involvement (p = 0.059) and age at implantation (p = 0.063) were borderline significant predictors. Patients with syncope at time of diagnosis were significantly less likely to receive ICD therapy (p = 0.02). Invasive electrophysiological testing was not significantly associated with appropriate ICD therapy.ConclusionIn our cohort of patients with ARVC, history of heart failure was a significant predictor of appropriate ICD therapy, whereas left ventricular involvement and age at time of ICD implantation were of borderline significance. These predictors should be tested in larger prospective cohorts to optimize ICD therapy in this rare cardiomyopathy.
Highlights
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetically transmitted disease prone to ventricular arrhythmias
It has been previously demonstrated that ARVC patients who have extensive right ventricular dilatation and/or left ventricular involvement confirmed by echocardiography or cardiac magnetic resonance imaging, and those who had hemodynamic significant ventricular tachycardia, as well as patients with affected relatives having survived a sudden cardiac death (SCD) are at increased risk and may benefit from implantable cardioverter defibrillator (ICD) therapy [16,17,18,19,20]
Patient charts were meticulously screened for past history of heart failure, which was defined by the presence of the following clinical features: symptoms of heart failure described typically by shortness of breath according to the New York Heart Association (NYHA) functional classification and/or fatigue; physical signs of fluid retention, such as pulmonary congestion or ankle swelling; and objective evidence of a structural or functional cardiac abnormality as cardiomegaly, third heart sound, abnormality on the echocardiogram, and elevated natriuretic peptide concentration
Summary
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare genetically transmitted disease prone to ventricular arrhythmias. We investigated the clinical, echocardiographical and electrophysiological predictors of appropriate implantable cardioverter defibrillator (ICD) therapy in patients with ARVC. Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inheritable cardiomyopathy with typically autosomal dominant inheritance pathologically characterized by progressive fibrofatty replacement of the right ventricular myocardium. This type of cardiomyopathy is a major cause of sudden cardiac death in young adults and athletes [1,2,3,4,5,6,7,8,9]. Implantable cardioverter defibrillators (ICD) are increasingly utilized in patients with ARVC who have survived SCD or have developed ventricular tachyarrhythmias associated with syncope [13]. This study aimed to determine clinical, echocardiographical and electrophysiological predictors of appropriate ICD therapy in our cohort of ARVC patients
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