Aims: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a progressive inherited cardiac disease. Early detection of disease and risk stratification remain challenging due to heterogeneous phenotypic expression. The standard configuration of the 12 lead electrocardiogram (ECG) might be insensitive to identify subtle ECG abnormalities.
View Article and Find Full Text PDFElectrical activity of the myocardium is recorded with the 12-lead ECG. ECG simulations can improve our understanding of the relation between abnormal ventricular activation in diseased myocardium and body surface potentials (BSP). However, in equivalent dipole layer (EDL)-based ECG simulations, the presence of diseased myocardium breaks the equivalence of the dipole layer.
View Article and Find Full Text PDFIntroduction: Inherited cardiomyopathies are associated with a broad spectrum of potentially lethal phenotypes characterized by structural and electrical myocardial remodeling. Increased awareness and genetic cascade screening lead to more genotype-positive, yet phenotype-negative individuals to be evaluated and followed up. The predictive value of genetic testing is hampered by incomplete penetrance and high variability in disease onset, progression and severity.
View Article and Find Full Text PDFObjectives: The goal of this study was to describe characteristics, cascade screening results, and predictors of adverse outcome in pediatric-onset arrhythmogenic right ventricular cardiomyopathy (ARVC).
Background: Although ARVC is increasingly recognized in children, pediatric ARVC cohorts remain underrepresented in the literature.
Methods: This study included 12 probands with pediatric-onset ARVC (aged <18 years at diagnosis) and 68 pediatric relatives (aged <18 years at first evaluation) referred for cascade screening.