Highlights
- •Brugada syndrome is a genetic disorder with increased risk of sudden cardiac death.
- •Brugada-related sudden cardiac death is due to ventricular tachycardia/fibrillation.
- •Typical ECG shows pseudo-right bundle branch block and coved ST elevation in V1–V2.
- •The Brugada ECG pattern is often intermittent, thus hindering the diagnosis.
- •~1.0% of adults have typical ECG pattern but stay asymptomatic for their lifetime.
Abstract
Brugada syndrome (BrS) is one of the commonest inherited primary arrhythmia syndromes
typically presenting with arrhythmic syncope or sudden cardiac death (SCD) due to
polymorphic ventricular tachycardia and ventricular fibrillation precipitated by vagotonia
or fever in apparently healthy adults, less frequently in children. The prevalence
of the syndrome (0.01%–0.3%) varies among regions and ethnicities, being the highest
in Southeast Asia.
BrS is diagnosed by the “coved type” ST-segment elevation ≥ 2 mm followed by a negative T-wave in ≥1 of the right precordial leads V1–V2. The typical electrocardiogram in BrS is often concealed by fluctuations between
normal, non-diagnostic and diagnostic ST-segment pattern in the same patient, thus
hindering the diagnosis.
Presently, the majority of BrS patients is incidentally diagnosed, and may remain
asymptomatic for their lifetime. However, BrS is responsible for 4–12% of all SCDs
and for ~20% of SCDs in patients with structurally normal hearts. Arrhythmic risk is the highest
in SCD survivors and in patients with spontaneous BrS electrocardiogram and arrhythmic
syncope, but risk stratification for SCD in asymptomatic subjects has not yet been
fully defined. Recent achievements have expanded our understanding of the genetics
and electrophysiological mechanisms underlying BrS, while radiofrequency catheter
ablation may be an effective new approach to treat ventricular tachyarrhythmias in
BrS patients with arrhythmic storms.
The present review summarizes our contemporary understanding and recent advances in
the inheritance, pathophysiology, clinical assessment and treatment of BrS patients.
Keywords
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Article info
Publication history
Published online: June 20, 2017
Accepted:
June 16,
2017
Received in revised form:
May 18,
2017
Received:
February 16,
2017
Identification
Copyright
© 2017 Published by Elsevier B.V. on behalf of European Federation of Internal Medicine.
