Brugada Syndrome

Impact of clinical and genetic findings on the management of young patients with Brugada syndrome

“…The Brugada Syndrome (BrS) is an arrhythmogenic disease 1 , with a heterogeneous genetic background [2][3][4][5] , usually diagnosed during adulthood. Spontaneous type 1 ECG pattern and symptoms are predictive risk factors for life-threatening arrhythmias (LTA) in adults 2 but, given the rarity of BrS in the pediatric population, risk stratification in the young is difficult.…”

Brugada syndrome: current diagnostics, epidemiology, genetic data and novel mechanisms (RCD code: V-1A.1)

“…The prevalence of BrS ECG patterns in different regions of the world (selected studies) is shown in Table 1 [11]. Interestingly, the average presence of spontaneous type 1 BrS ECG pattern among East Asian population investigated in the Healthy Aging Longitudinal Study in Taiwan (HALST) cohort was similar to world-wide prevalence (0.077 vs. 0.07%), but saddleback BrS ECG pattern (type 2 and type 3 according to previous classification) was over 10 times more frequent in this Asian population when compared to the world-wide frequency (3.24 vs. 0.28%) [12].…”

The Use of Implantable Cardioverter-defibrillators in the Prevention of Sudden Cardiac Death: A Focus on Congenital Heart Disease and Inherited Arrhythmia Syndromes

“…62 More than 12 responsible genes have been identified; around one-third of patients with diagnostic ECG findings have an identifiable pathogenic mutation. 63,64 There are three ECG morphologies associated with BrS. Type 1 morphology is characterized by the presence of at least 2 mm of coved ST segment elevation in at least one right precordial lead (V1-V4).…”