Brugada syndrome
Brugada syndrome is an inherited sodium channelopathy causing a characteristic coved ST elevation in the right precordial leads and a risk of polymorphic ventricular tachycardia and sudden death, typically during rest, sleep, or fever. Management centers on an ICD for high-risk patients, fever control, and avoidance of provoking drugs.
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What this article covers
- Overview
Definition: Brugada syndrome is an inherited arrhythmia disorder, usually a sodium channelopathy, defined by a type 1 Brugada ECG pattern (coved ST elevation in the right precordial leads) with a…
- Epidemiology
More common in men and in Southeast Asian populations.
- Risk factors
SCN5A and related pathogenic mutations and family history of sudden death.
- Pathophysiology
Reduced sodium current: loss-of-function sodium channel mutations reduce the depolarizing current, most prominently in the right ventricular outflow tract epicardium.
- Clinical features
Syncope from self-terminating polymorphic VT.
- Differential diagnosis
Brugada ECG phenocopies: electrolyte disturbance, ischemia, or drugs producing a similar coved pattern, distinguished because the pattern normalizes once the reversible cause is corrected, unlike the…
- Diagnosis
Establish by a type 1 Brugada ECG pattern, spontaneous or drug-induced, with clinical or family criteria: the diagnostic basis is a coved ST-segment elevation of at least 2 mm followed by a negative…
- Management
First-line, risk-stratify and place an ICD in high-risk patients: implant an ICD for survivors of cardiac arrest and for a spontaneous type 1 pattern with syncope, as definitive protection.
- Complications
Polymorphic ventricular tachycardia and fibrillation.
- Prognosis
Good for asymptomatic patients with only a drug-induced pattern.
- Prevention
ICD for high-risk patients. Aggressive fever control with antipyretics (acetaminophen 650 to 1000 mg every 6 hours as needed).
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