Brugada Syndrome: Current Practices in Diagnosis, Prognosis, and Treatment

Abstract

<p>Brugada syndrome (BrS) is a hereditable syndrome, first reported in 1992, characterized by right bundle branch block and an uncommon form of ST-T wave elevation in the V1 and V2 leads and associated with risk of sudden cardiac death (SCD) arising from polymorphic ventricular tachyarrhythmias. BrS is an autosomal dominant inherited condition; however, more than 50% of BrS cases may be sporadic. Approximately 20% to 25% of BrS cases originate from loss of function mutations in the SCN5A cardiac sodium channel.</p><p>The diagnosis of BrS is mainly based on electrocardiogram. SCD due to ventricular fibrillation can be the first clinical presentation of BrS. The insertion of an implantable cardioverter-defibrillator remains the only approved effective measure to prevent SCD in BrS patients. Risk stratification in BrS is still challenging. Because the role of electrophysiological study (EPS) for estimating prognosis in BrS patients has been controversial, but the expert consensus published in 2013 (Priori et al, 2013) considered the performance of EPS, class IIb. Future randomized studies focused on risk stratification and the value of radiofrequency ablation in BrS patients are needed.</p><p>This review provides a succinct general overview of BrS focusing on<strong> </strong>current practices in diagnosis, prognosis, and treatment.</p>