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

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 th...

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Bibliographic Details
Published in:Journal of the American College of Cardiology 2018-08, Vol.72 (9), p.1046-1059
Main Authors: Brugada, Josep, Campuzano, Oscar, Arbelo, Elena, Sarquella-Brugada, Georgia, Brugada, Ramon
Format: Article
Language:English
Subjects:
Ablation
Age
Brugada Syndrome - diagnosis
Brugada Syndrome - etiology
Brugada Syndrome - therapy
Cardiac arrhythmia
Cardiology
Cardiovascular diseases
Catheters
Causation
Defibrillators
Electrocardiography
Family medical history
Fibrillation
Genetic diversity
Genetic variance
Genetics
Heart
Humans
Males
Medical instruments
Mortality
Older people
Patients
Population
Radio frequency
Risk management
Sexes
Sodium
Studies
Ventricle
Ventricular fibrillation
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Summary: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 V to V ), 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.
ISSN:0735-1097
1558-3597
DOI:10.1016/j.jacc.2018.06.037