(A) Genotype and phenotype is defined according to the legend inset. Open symbols represent subjects with a negative genotype and phenotype. An arrow marks the proband (III-3). Symbols with a question mark represent subjects with unknown phenotype. The QTc interval, the age of onset and the treatment are reported below each subject symbol (abbreviations: Mex, mexiletine; Met, Metoprolol; LCD, left cardiac sympathetic denervation; ICD, implantable cardioverter defibrillator; SCD, sudden cardiac death). (B) Representative ECG traces from four genotype positive family members.