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J Am Coll Cardiol. 2018 Aug 28;72(9):1046-1059. doi: 10.1016/j.jacc.2018.06.037.

Present Status of Brugada Syndrome: JACC State-of-the-Art Review.

Author information

1
Institut Clínic Cardiovascular (ICCV), Hospital Clínic, Universitat de Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; Arrhythmias Unit, Hospital Sant Joan de Déu, University of Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain. Electronic address: jbrugada@clinic.cat.
2
Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain; Cardiovascular Genetics Center, Institut d'Investigació Biomèdica Girona (IDIBGI), University of Girona, Girona, Spain; Medical Science Department, School of Medicine, University of Girona, Girona, Spain.
3
Institut Clínic Cardiovascular (ICCV), Hospital Clínic, Universitat de Barcelona, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Spain; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain.
4
Arrhythmias Unit, Hospital Sant Joan de Déu, University of Barcelona, Barcelona, Spain; Medical Science Department, School of Medicine, University of Girona, Girona, Spain.
5
Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Madrid, Spain; Cardiovascular Genetics Center, Institut d'Investigació Biomèdica Girona (IDIBGI), University of Girona, Girona, Spain; Medical Science Department, School of Medicine, University of Girona, Girona, Spain; Cardiology Service, Hospital Josep Trueta, Girona, Spain.

Abstract

The Brugada syndrome is an inherited disorder associated with risk of ventricular fibrillation and sudden cardiac death in a structurally normal heart. Diagnosis is based on a characteristic electrocardiographic pattern (coved type ST-segment elevation ≥2 mm followed by a negative T-wave in ≥1 of the right precordial leads V1 to V2), observed either spontaneously or during a sodium-channel blocker test. The prevalence varies among regions and ethnicities, affecting mostly males. The risk stratification and management of patients, principally asymptomatic, still remains challenging. The current main therapy is an implantable cardioverter-defibrillator, but radiofrequency catheter ablation has been recently reported as an effective new treatment. Since its first description in 1992, continuous achievements have expanded our understanding of the genetics basis and electrophysiological mechanisms underlying the disease. Currently, despite several genes identified, SCN5A has attracted most attention, and in approximately 30% of patients, a genetic variant may be implicated in causation after a comprehensive analysis.

KEYWORDS:

Brugada syndrome; arrhythmias; genetics; sudden cardiac death

PMID:
30139433
DOI:
10.1016/j.jacc.2018.06.037

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