What is the management approach for a condition referred to as 'Bers' on an Electrocardiogram (ECG)?

Management of Brugada Syndrome (BrS) ECG Pattern

If a Type 1 Brugada ECG pattern is identified, immediate referral to an electrophysiologist is mandatory regardless of symptoms, as this represents a potentially life-threatening inherited arrhythmia syndrome with up to 10% annual mortality if untreated. 1, 2

Initial ECG Confirmation and Differentiation

• Verify correct lead placement and repeat the ECG if the pattern is unclear, specifically placing V1 and V2 in the 2nd or 3rd intercostal space (high precordial leads) to unmask or confirm the Type 1 pattern 1

• Distinguish true Brugada from benign early repolarization using the Corrado index: measure ST-segment elevation at the J-point (STJ) and 80 ms after (ST80). A STJ/ST80 ratio >1 indicates downsloping ST-segment consistent with Brugada, while ratio <1 indicates upsloping early repolarization seen in athletes 1, 3

• Confirm Type 1 diagnostic criteria: coved rSr' pattern with ST-segment elevation ≥2 mm and terminal T-wave inversion in leads V1-V3 1, 4

Critical Clinical Assessment

• Obtain detailed personal history focusing on syncope (especially during sleep or rest), aborted cardiac arrest, seizure-like episodes, or palpitations, as these symptoms predict life-threatening arrhythmias (LTA) 1, 5

• Document family history of sudden cardiac death before age 50, unexplained drowning, motor vehicle accidents, or known Brugada syndrome, as this is inherited in autosomal dominant pattern with variable penetrance 1

• Identify fever or recent febrile illness, as fever is a major trigger for ventricular arrhythmias in 27-33% of BrS patients and increases sudden cardiac arrest risk 1, 5

• Review all medications for sodium channel blockers (antiarrhythmics, certain antidepressants, local anesthetics) and QT-prolonging drugs, as these can unmask or worsen the ECG pattern 1

Risk Stratification for Sudden Cardiac Death

High-risk patients requiring immediate ICD consideration:

• Spontaneous Type 1 ECG pattern (not drug-induced) with history of cardiac arrest or documented ventricular fibrillation 5
• Spontaneous Type 1 pattern with syncope of suspected arrhythmic origin 1, 5
• Both spontaneous Type 1 ECG and symptoms at diagnosis predict shortest time to LTA events 5

Intermediate-risk patients:

• Spontaneous Type 1 pattern in asymptomatic patients discovered incidentally 5
• Drug-induced Type 1 pattern with positive family history of sudden death 1

Lower-risk patients:

• Drug-induced Type 1 pattern in asymptomatic patients with negative family history 5

Immediate Management Actions

• Initiate continuous cardiac monitoring until risk stratification is complete, as ventricular arrhythmias occur predominantly during sleep or rest due to enhanced vagal tone 1, 4

• Treat fever aggressively with antipyretics in any patient with suspected or confirmed BrS, as fever is a critical modifiable trigger for sudden death 1, 5

• Discontinue all sodium channel blocking medications and avoid QT-prolonging drugs 1

• Perform echocardiography to confirm absence of structural heart disease, which is required for BrS diagnosis 1

Definitive Treatment Decisions

Symptomatic patients (cardiac arrest survivors or syncope):

• Implantable cardioverter-defibrillator (ICD) is the only proven therapy to prevent sudden death and is the treatment of choice 6, 2
• Note that 41% of pediatric patients experience ICD-related complications, requiring careful risk-benefit discussion 5

Asymptomatic patients with spontaneous Type 1 pattern:

• Consider quinidine (hydroquinidine) therapy as an alternative to ICD or for patients refusing ICD, as 73% remain asymptomatic on this therapy 5
• ICD may be considered based on electrophysiology study results and shared decision-making 4

Asymptomatic patients with drug-induced pattern only:

• Conservative management with drug avoidance and fever management is appropriate 4
• Aggressive fever treatment protocols should be established 5

Genetic Testing and Family Screening

• Perform genetic testing for SCN5A mutations in all probands, as 47-55% will be genotype-positive and all patients with LTA events in pediatric studies were genotype-positive 1, 5

• Screen first-degree relatives with ECG (including high precordial leads) and consider sodium channel blocker challenge if initial ECG is normal 1

• Recognize genetic heterogeneity: only 20-26% of BrS patients have identifiable SCN5A mutations, so negative genetic testing does not exclude diagnosis 1, 6

Critical Pitfalls to Avoid

• Never dismiss asymptomatic patients with spontaneous Type 1 pattern as benign—they remain at risk for sudden death and require electrophysiology referral 1, 5

• Do not rely on antiarrhythmic drugs (beta-blockers, amiodarone) for prevention, as these have no proven benefit in prolonging survival 6, 2

• Avoid misdiagnosing athletic early repolarization as Brugada—use the Corrado index systematically 1, 3

• Never overlook fever as a trigger—establish clear protocols for aggressive antipyretic use in all confirmed BrS patients 1, 5