Brugada Syndrome

Brugada Syndrome

BrugadaECGPedia
Short Question Brugada Syndrome
What is Brugada Syndrome? A genetic arrhythmia disorder with characteristic ECG changes and risk of sudden cardiac death.
Most common genetic mutation? SCN5A gene mutation.
Typical inheritance pattern? Autosomal dominant.
Typical ECG hallmark? Coved-type ST-segment elevation in V1–V3.
Which ECG lead placement can unmask the pattern? High intercostal placement of V1–V2.
Most common arrhythmia in Brugada Syndrome? Polymorphic ventricular tachycardia or VF.
Drug used for diagnostic challenge? Ajmaline or flecainide.
Which gender is more affected? Males (about 8:1 male-to-female ratio).
Most dangerous complication? Sudden cardiac death.
Definitive treatment for high-risk patients? Implantable cardioverter-defibrillator (ICD).
Common trigger for arrhythmia? Fever.
What should be avoided in Brugada Syndrome? Sodium channel-blocking drugs unless for diagnosis.
Region of heart primarily affected? Right ventricular outflow tract (RVOT).
Type 1 Brugada ECG pattern description? Coved ST elevation ≥2 mm with inverted T wave in V1–V3.
Geographical prevalence? More common in Southeast Asia and Japan.
First described by? Pedro and Josep Brugada in 1992.
Can Brugada be asymptomatic? Yes, many cases are found incidentally on ECG.
Prognosis without treatment in high-risk patients? Poor, due to high risk of sudden death.
Can ablation help? Epicardial ablation of RVOT substrate may reduce arrhythmia risk.
Role of family screening? Recommended for first-degree relatives.
1) Brugada syndrome is best described as:
A) Structural cardiomyopathy with LV dilation
B) Inherited primary electrical disease with ST elevation in V1–V3
C) Pericardial inflammatory disorder
D) Congenital cyanotic heart disease
Brugada is a channelopathy in structurally normal hearts with malignant ventricular arrhythmia risk.
2) The diagnostic (Type 1) ECG pattern shows:
A) Diffuse concave ST elevation with PR depression
B) Coved ST elevation ≥2 mm in V1–V2 with inverted T wave
C) ST depression V5–V6 with tall T waves
D) Delta waves with short PR
Type 1 is “coved” ST elevation in right precordial leads with T-wave inversion.
3) Which leads are most important for detecting Brugada pattern?
A) V1–V3 (especially V1–V2)
B) II, III, aVF
C) I, aVL, V6
D) aVR only
Right precordial leads reflect RV outflow tract/anteroseptal electrical activity.
4) Which maneuver increases sensitivity for Brugada ECG?
A) Hyperventilation
B) Place V1–V2 in 2nd–3rd intercostal space
C) Left lateral decubitus
D) Exercise stress testing
High right precordial placement may unmask Type 1 morphology.
5) Which factor commonly unmasks or worsens the Brugada ECG?
A) Hypothermia
B) Fever
C) Hyperkalemia
D) Hyperthyroidism
Fever reduces sodium channel availability and can precipitate VT/VF.
6) The most commonly implicated gene in Brugada syndrome is:
A) SCN5A
B) KCNQ1
C) MYH7
D) PKP2
SCN5A encodes the cardiac sodium channel α-subunit; variants in ~20% of cases.
7) The inheritance pattern is typically:
A) Autosomal dominant with variable penetrance
B) Autosomal recessive
C) X-linked recessive
D) Mitochondrial
Family history may be subtle due to incomplete penetrance.
8) Definitive therapy for high-risk Brugada syndrome:
A) Beta-blockers
B) Implantable cardioverter-defibrillator (ICD)
C) AV node ablation
D) Calcium channel blockers
ICD prevents sudden cardiac death in survivors of arrest or symptomatic high-risk patients.
9) Acute treatment for electrical storm in Brugada:
A) Amiodarone bolus
B) Isoproterenol infusion
C) Adenosine
D) Digoxin
Isoproterenol increases ICa and heart rate, stabilizing the action potential dome.
10) Which oral drug reduces recurrent VT/VF or ICD shocks in Brugada?
A) Quinidine
B) Flecainide
C) Propafenone
D) Procainamide
Quinidine blocks Ito, restoring the epicardial AP dome and reducing arrhythmogenic substrate.
11) “Brugada pattern” vs “Brugada syndrome”:
A) Pattern requires symptoms; syndrome does not
B) Pattern = ECG only; Syndrome = ECG + clinical criteria (e.g., syncope, VF)
C) Terms are interchangeable
D) Pattern needs genetic confirmation
Syndrome requires phenotype (events/risk markers) in addition to the ECG.
12) A common clinical trigger for malignant arrhythmia in Brugada is:
A) Febrile illness
B) Exercise in heat
C) Hypoglycemia
D) Orthostatic stress
Treat fevers promptly with antipyretics to avoid arrhythmic events.
13) Which class of drugs should generally be avoided in Brugada syndrome?
A) Sodium channel blockers (e.g., flecainide)
B) ACE inhibitors
C) Statins
D) Thiazide diuretics
Sodium-channel blockers can unmask/worsen the Brugada pattern and provoke arrhythmias.
14) Typical timing of arrhythmic events in Brugada:
A) During maximal exercise
B) At rest or during sleep
C) Immediately postprandial only
D) Strictly midday
Vagal predominance at night favors arrhythmogenesis in Brugada.
15) Provocative drug testing (e.g., ajmaline/flecainide) is used to:
A) Unmask a Type 1 pattern when baseline is non-diagnostic
B) Treat electrical storm
C) Assess LV systolic function
D) Diagnose pericarditis
Testing is performed under monitoring to reveal diagnostic morphology.
16) Which ECG placement tip improves detection of Brugada type 1?
A) Move V5–V6 to right chest
B) Place V1–V2 higher (2nd–3rd intercostal spaces)
C) Put limb leads on chest
D) Reverse all precordial leads
High right precordial positions increase sensitivity.
17) Brugada syndrome hearts are typically:
A) Structurally normal on imaging
B) Severely dilated
C) Markedly hypertrophied
D) With global scar
It’s a primary electrical disease (channelopathy), not a primary structural cardiomyopathy.
18) A common symptom suggestive of Brugada syndrome is:
A) Exertional angina
B) Syncope at rest/night
C) Orthopnea
D) Claudication
Syncope, nocturnal agonal respirations, or palpitations may herald malignant ventricular arrhythmias.
19) In Brugada electrical storm, besides isoproterenol, which therapy can help?
A) Quinidine
B) Class IC agents
C) Diltiazem
D) Digoxin
Quinidine reduces Ito and is useful for recurrent events/ICD shocks.
20) Which bedside advice is MOST appropriate for known Brugada patients?
A) Treat fever aggressively and avoid Brugada-contraindicated drugs
B) Encourage sauna exposure
C) Stop all exercise
D) Start over-the-counter sodium blockers
Fever control and medication vigilance are key to preventing arrhythmic events.
# Brugada Syndrome
1Brugada Syndrome is a genetic disorder affecting cardiac sodium channels.
2Most cases are linked to mutations in the SCN5A gene.
3Characterized by coved-type ST-segment elevation in V1–V3 on ECG.
4Often presents with syncope or sudden cardiac arrest, especially during rest or sleep.
5More common in men than women, with higher prevalence in Southeast Asia.
6Triggers include fever, certain medications, and electrolyte imbalances.
7Type 1 ECG pattern is diagnostic; types 2 and 3 require provocation testing.
8Ajmaline or flecainide challenge can unmask concealed Brugada patterns.
9Risk stratification is based on symptoms and family history of sudden cardiac death.
10ICD implantation is the mainstay of therapy in high-risk patients.
11Quinidine can be used in patients who are not ICD candidates or have recurrent ICD shocks.
12Catheter ablation may target arrhythmogenic substrate in the RV outflow tract.
13Febrile illnesses should be treated aggressively to prevent arrhythmic events.
14Certain drugs such as Class I antiarrhythmics, tricyclic antidepressants, and lithium should be avoided.
15ECG changes may be intermittent and influenced by autonomic tone.
16Family screening is essential for early detection in asymptomatic carriers.
17The arrhythmia mechanism often involves phase 2 reentry in the RV epicardium.
18Polymorphic ventricular tachycardia or VF are typical terminal rhythms in events.
19ECG lead placement modification (high V1–V3) can improve detection.
20Brugada phenocopies can mimic ECG changes but are caused by reversible conditions.

Brugada syndrome, Brugada type 1 ECG, SCN5A mutation, RVOT ablation, ajmaline challenge, flecainide challenge, fever unmasking Brugada, ICD in Brugada, sudden cardiac death risk,,LSI/Supporting Terms: coved ST elevation, V1–V3 leads, sodium channelopathy, polymorphic VT, ventricular fibrillation, quinidine therapy, Brugada phenocopy, high intercostal V1–V2

ECG patterns

There are three main types of ECG patterns linked to Brugada syndrome, primarily seen in the right chest leads (V1-V3): 

  • Type 1 (diagnostic): Features a coved-type ST-segment elevation of 2 mm or more, followed by a negative T wave. This pattern is considered diagnostic.
  • Type 2: Shows a saddleback-shaped ST-segment elevation of 2 mm or more at the high take-off, a trough with at least 1 mm of ST elevation, and a positive or biphasic T wave.
  • Type 3: Displays either a saddleback or coved-type ST-segment elevation less than 1 mm. 

Note: Only the Type 1 ECG pattern confirms Brugada syndrome. Type 2 and Type 3 patterns may require further tests, like a drug challenge, to reveal a Type 1 pattern and confirm the diagnosis. 

ECG lead placement

Sometimes, placing the right chest leads (V1-V3) higher on the chest (in the second or third intercostal space) can help reveal a subtle or hidden Brugada ECG pattern, especially Type 1. 

Other ECG characteristics

Other ECG findings that may be present but are not specifically diagnostic include: 

  • Incomplete right bundle branch block (RBBB)
  • Prolonged PR interval
  • Left axis deviation
  • Atrial fibrillation 

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