title: Pathogenesis and management of Brugada syndrome.
abstract: Brugada syndrome is an inherited disease characterized by an increased risk of sudden cardiac death owing to ventricular arrhythmias in the absence of structural heart disease. Since the first description of the syndrome >20 years ago, considerable advances have been made in our understanding of the underlying mechanisms involved and the strategies to stratify at-risk patients. The development of repolarization-depolarization abnormalities in patients with Brugada syndrome can involve genetic alterations, abnormal neural crest cell migration, improper gap junctional communication, or connexome abnormalities. A common phenotype observed on the electrocardiogram of patients with Brugada syndrome might be the result of different pathophysiological mechanisms. Furthermore, risk stratification of this patient cohort is critical, and although some risk factors for Brugada syndrome have been frequently reported, several others remain unconfirmed. Current clinical guidelines offer recommendations for patients at high risk of developing sudden cardiac death, but the management of those at low risk has not yet been defined. In this Review, we discuss the proposed mechanisms that underlie the development of Brugada syndrome and the current risk stratification and therapeutic options available for these patients.
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Brussels, Laarbeeklaan 101, 1090 Brussels, Belgium.|Cardiology Department, University Hospital Erasme, Route de Lennik 808, 1070 Brussels, Belgium.
authors.forename: Juan
authors.initials: J
authors.lastname: Sieira
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Brussels, Laarbeeklaan 101, 1090 Brussels, Belgium.|Cardiovascular Division, University Hospital "Paolo Giaccone", Via Del Vespro 127. 90127 Palermo, Italy.
authors.forename: Gregory
authors.initials: G
authors.lastname: Dendramis
authors.affiliation: Heart Rhythm Management Centre, UZ Brussel-VUB, Brussels, Laarbeeklaan 101, 1090 Brussels, Belgium.
authors.forename: Pedro
authors.initials: P
authors.lastname: Brugada
mesh_terms: D000889:Anti-Arrhythmia Agents / Q000627:therapeutic use; D053840:Brugada Syndrome / Q000150:complications / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy*; D017115:Catheter Ablation; D016757:Death, Sudden, Cardiac / Q000209:etiology; D017147:Defibrillators, Implantable; D004562:Electrocardiography; D055724:Electrophysiological Phenomena; D006801:Humans; D011802:Quinidine / Q000627:therapeutic use; D018570:Risk Assessment; D012307:Risk Factors
chemical_list: D000889:Anti-Arrhythmia Agents; D011802:Quinidine