Brugada syndrome is a genetic cardiac condition characterized by ST-segment elevation in the right precordial leads and risk of sudden cardiac death. It is caused by mutations resulting in loss of function of cardiac sodium channels. Diagnosis requires a characteristic ECG pattern plus symptoms like syncope or family history of sudden cardiac death. For those with a history of ventricular arrhythmias or syncope, an ICD is recommended for prevention of sudden death.

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BRUGADA SYNDROME
INTRODUCTION
Recently discovered
Pedro and Brugada described the syndrome in 1992 - ST-segment elevation in the right
precordial leads and a high incidence of sudden death with structurally normal hearts
Genetic disorder with variable expression
Channelopathy
IMPORTANCE OF DIAGNOSING
Autosomal Dominant
Increased risk of ventricular tachyarrhythmias and SCD
Family to be screened
BRUGADA PATTERN - Patients with typical ECG features but no clinical criteria
BRUGADA SYNDROME - patients with typical ECG features and other clinical criteria
EPIDEMIOLOGY
Asian esp. Japan
ECG pattern is dynamic and may be concealed
Median age 41years
In one series, 4% of all sudden deaths and 20% sudden death in structurally normal heart
M>F
PATHOGENESIS
Loss of function mutations in SCN5A
Additional genes implicated - Cardiac calcium, potassium channel
Chromosome 3p
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by Dr. Eashan Srivastava

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Benjamin Franklin
Reduce sodium inflow currents, thereby reducing the duration of normal action potentials.
Ventricular arrhythmias – due to heterogeneity of myocardial refractory periods in
the right ventricle. Heterogeneity arises from the presence of both normal and abnormal sodium
channels in the same tissue.
C/F
1. Sudden cardiac arrest
2. Syncope
3. Palpitations
4. Nocturnal Agonal Respiration-Gasping breaths during sleep, may represent aborted
Arrhythmia. Ominous symptom & equivalent of syncope/ventricular arrhythmia
TRIGGERS
● Autonomic disturbances – Increased Sympathetic tone
● Fever
● Drugs-Beta blockers, TCAs, lithium, alcohol and cocaine toxicity, Sodium channel
blocker e.g.: Flecainide
● Pacing, manoeuvres increased alpha adrenergic tone
● Hypo/hyper Kalemia and hypercalcemia
ECG Patterns
Pseudo RBBB with ST elevation in V1-V3
TYPES
Type 1 - ​elevated ST segment (>2mm) descending with upward convexity to inverted T wave
(“coved type”)
Type 2 -​ ST segment elevated (>2mm) saddle back configuration
Type 3 -​ Morphology either Type 1 or Type 2 but ST elevation < 2mm
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by Dr. Eashan Srivastava

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Fluctuations in ECG pattern may occur
More prone to atrial tachyarrhythmias especially atrial fibrillation
Patients with AF indicate greater disease severity
DIAGNOSTIC TESTING AND RISK STRATIFICATION
Drug testing
Requires continuous ECG monitoring
Brugada ECG can be unmasked by sodium channel blockers
Not recommended in patients who have documented VT/VF/unexplained syncope
Flecainide – 2 mg/kg over 10 minutes intravenously or 400 mg PO
Termination of drug challenge
• Development of a diagnostic Brugada ECG pattern
• ≥2 mm increase in ST segment elevation in patients with a type 2 Brugada ECG
pattern
• Development of VPC or other arrhythmias
• Widening of the QRS ≥30 percent above baseline
Signal Averaged ECG
Computerized technique for detecting subtle abnormalities in the ECG that are not visible
to the naked eye.
Electrophysiological Testing
Inducible ventricular arrhythmia during EP testing predictor of future arrhythmic events
Genetic testing
• Sequencing of SCN5A
• Family screening
DIAGNOSTIC CRITERIA
Diagnosis = clinically significant events + typical ECG events
EVENTS=
• Documented VT/VF
• Family history of SCD at less than 45 years of age
• Family history of Brugada pattern ECG changes
• Inducible VT during EPS
• Unexplained syncope
• Nocturnal agonal respiration
DIFFERENTIAL DIAGNOSIS​​ ​BRUGADA PATTERN
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• Atypical RBBB
• Arrhythmogenic right ventricular cardiomyopathy
• Early repolarization
• Acute pericarditis
•Acute myocardial ischemia or infarction
• Hypothermia
POOR PROGNOSTIC FACTORS
● Aborted SCD
● Syncope
● Type 1 ECG
● AF have a higher risk of VF
● Male
● family history of SCA
TREATMENT = ICD
Brugada pattern do not require any Therapy
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by Dr. Eashan Srivastava

5. Wise men don't need advice. Fools won't take it
Benjamin Franklin
- Created with love
by Dr. Eashan Srivastava

6. Wise men don't need advice. Fools won't take it
Benjamin Franklin
Focal RF ablation of the Ventricular premature beats
Drugs
● Quinidine
● Amiodarone is the most effective agent for the prevention of ventricular
tachyarrhythmias
● Tedisamil - experimental antiarrhythmic agent which blocks ITO is a therapeutic
candidate.
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by Dr. Eashan Srivastava