Paola G. Meregalli

TL;DR: In the largest series of Brugada syndrome patients thus far, event rates in asymptomatic patients were low and gender, familial history of SCD, inducibility of ventricular tachyarrhythmias during electrophysiological study, and the presence of an SCN5A mutation were not predictive of arrhythmic events.

TL;DR: Clinical and experimental studies are reviewed as they provide evidence to support or disprove the two hypotheses on the mechanism of Brugada syndrome that currently receive the widest support: (1) nonuniform abbreviation of right ventricular epicardial action potentials ("repolarization disorder"), (2) conduction delay in theright ventricular outflow tract ("depolarization Disorder").

TL;DR: In the largest population of children affected by Brugada syndrome described to date, fever represented the most important precipitating factor for arrhythmic events, and as in the adult population, the risk of arrhythmmic events was higher in previously symptomatic patients and in those displaying a spontaneous type I ECG.

TL;DR: The results suggest that SCN5A mutations are not directly causal to the occurrence of a BrS-ECG+ and that genetic background may play a powerful role in the pathophysiology of BrS.

TL;DR: In loss-of-function SCN5A channelopathies, patients carrying T and M(inactive) mutations develop a more severe phenotype than those with M( active) mutations, associated with more severe conduction disorders.