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Highly malignant disease in childhood-onset arrhythmogenic right ventricular cardiomyopathy: study design and summary results. In a paediatric ARVC cohort, childhood-onset ARVC was found in 40% of all patients with a definite diagnosis. There was a 23% cumulative incidence of severecardiac events and half of them occurred in children ≤12 years of age. The ARVC penetrance in genotype positive paediatric relatives was 18%.
