LAUSR

SEE PAGE 1756 B rugada syndrome (BrS) is a genetic arrhythmic disease with a typical electrocardiographic pattern and an increased risk of sudden cardiac death (SCD) (2,3). It was initially described as a pure electrical disorder caused by dysfunction of cardiac ion channels without structural heart disease, but imaging and pathology studies increasingly recognize the presence of structural abnormalities of the right ventricular outflow tract (RVOT) in many patients with BrS (4–7). Pathogenic variants in more than 20 genes encoding for proteins contributing to ion channels structure and function have been reported in BrS, although on the basis of clinical and cellular studies, current guidelines recommend a comprehensive genetic analysis of only the SCN5A gene (accounting for 25% to 30% of cases). In addition, results of genetic screening does not currently influence prognosis or treatment (2,3). The syndrome is 8 to 10 times more prevalent in men, and lethal arrhythmias usually first occur during adulthood, with a mean age of SCD presentation of 41 15 years. The mechanism of these sexand age-related differences is poorly understood. Hormonal influence has been implicated, as a higher concentration of testosterone has been noted in men with BrS and the male predominance among BrS patients is not observed in children younger than