Electrophysiologic study including electroanatomic mapping
Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is an inherited heart muscle disease characterized by specific right ventricular (RV) involvement and risk of arrhythmic sudden death [1-3]. Distinctive pathologic feature is RV myocardial atrophy with replacement by fibrofatty tissue [2-5]. The condition was initially believed to be a developmental defect of the RV myocardium, leading to the original designation of dysplasia ; this concept has evolved over the last 25 years into the current perspective of a genetically determined cardiomyopathy [6, 7]. The estimated prevalence of the disease in the general population ranges from 1 in 2,000 to 1 in 5,000. It affects men more frequently than women, with an approximate ratio of 3:1. Clinical manifestations develop most often between the second and fourth decade of life and usually are related to ventricular tachycardia of RV origin or ventricular fibrillation leading to cardiac arrest, mostly in young people [2, 4-6].Ventricular arrhythmias often are worse during or immediately after exercise, and participation in competitive athletics has been associated with an increased risk for sudden death [2, 4, 5]. Later in the disease evolution, progression of RV muscle disease and left ventricular involvement may result in right or biventricular heart failure . Clinical diagnosis of ARVC/D is often difficult due to the nonspecific nature of disease features and the broad spectrum of phenotypic manifestation, ranging from severe to concealed forms [9-11]. The first objective of management strategy is to assess arrhythmic risk and prevent sudden death. Therapeutic options include antiarrhythmic drugs, catheter ablation, and implantable cardioverter defibrillator (ICD) . © 2007 Springer-Verlag Italia.
Corrado, D; Daubert, J; Basso, C; Buja, G; Thiene, G
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