Abstract
Brugada syndrome, first described in 1992, is an autosomal dominant genetic disorder characterized by a distinctive ECG pattern and a risk of dangerous ventricular arrhythmias leading to sudden death. Most Brugada patients do not have clinical manifestations; instead, the disease is discovered incidentally through ECG abnormalities during routine checkups or by screening relatives of Brugada patients. For symptomatic patients, the clinical presentation is quite diverse with nonspecific symptoms including fainting (30%), nighttime breathing difficulties (12%), ventricular tachycardia/fibrillation episodes (6%), and sudden cardiac death (SCD) (6%). SCD may be the first manifestation of the disease without any preceding warning signs. As with other inherited arrhythmia disorders, there is high variability in the clinical phenotype among individual Brugada patients. Patients with Type 1 Brugada ECG pattern may not experience any arrhythmic events throughout their lifetime; however, there is still a certain probability of sudden death or other arrhythmic events occurring. This variability in clinical phenotype indicates that the pathophysiological mechanism of Brugada syndrome is relatively complex and still controversial. Up to now, with deeper understanding of the electrophysiological and genetic mechanisms as well as newly reported clinical evidence, there have been many changes in the diagnosis, risk stratification, and management of Brugada syndrome patients.
