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

Area of Science:

• Cardiology
• Genetics
• Electrophysiology

Background:

• Brugada syndrome is an inherited cardiac disorder.
• It is associated with ventricular fibrillation and sudden cardiac death.
• Diagnosis relies on characteristic ECG patterns and can be confirmed with sodium-channel blocker tests.

Purpose of the Study:

• To summarize current understanding of Brugada syndrome.
• To discuss diagnostic criteria and challenges in risk stratification.
• To review management strategies, including implantable cardioverter-defibrillators and radiofrequency catheter ablation.

Main Methods:

• Review of diagnostic criteria based on electrocardiographic patterns.
• Analysis of prevalence variations across populations.
• Examination of genetic underpinnings, focusing on SCN5A gene variants.
• Evaluation of current and emerging therapeutic interventions.

Main Results:

• Brugada syndrome affects structurally normal hearts, primarily males.
• ECG patterns (coved ST-segment elevation) are key diagnostic indicators.
• SCN5A gene variants are implicated in approximately 30% of patients.
• Radiofrequency catheter ablation shows promise as a new treatment.

Conclusions:

• Brugada syndrome management, especially for asymptomatic patients, remains challenging.
• Implantable cardioverter-defibrillators are the primary therapy.
• Radiofrequency catheter ablation is an emerging effective treatment option.
• Continued research into genetic and electrophysiological mechanisms is crucial.