Brugada syndrome
Pronunciation: broo-GAH-dah
An inherited channelopathy characterized by distinctive ECG patterns and increased risk of sudden cardiac death from ventricular arrhythmias.
Full Definition
Brugada syndrome is an inherited cardiac channelopathy caused by mutations in genes encoding cardiac sodium channels, most commonly SCN5A. It is characterized by a distinctive ECG pattern featuring coved-type ST elevation in leads V1-V3, often unmasked by sodium channel blockers like procainamide or flecainide. Patients are at increased risk for polymorphic ventricular tachycardia and sudden cardiac death, particularly during sleep or fever. The syndrome is more prevalent in Southeast Asian populations and young males. Management includes avoiding certain medications, fever control, and ICD implantation in high-risk patients.
Usage
Usage note: Named after the Brugada brothers who first described the syndrome; distinguish between Brugada pattern (ECG finding) and Brugada syndrome (clinical diagnosis).
In Context
- "The procainamide challenge test unmasked a type 1 Brugada pattern, confirming the diagnosis of Brugada syndrome." — Diagnostic test report
- "Family screening revealed Brugada syndrome in the proband's asymptomatic brother." — Genetic counseling report