Brugada Syndrome Caused by Mutation in CACNB2

Brugada syndrome caused by mutation in CACNB2 is a heart rhythm condition that makes the heart’s electrical system unstable. It can cause dangerous fast rhythms from the bottom chambers of the heart (ventricular arrhythmias). These rhythms can lead to fainting or sudden death, often during rest or sleep. The condition is diagnosed by a special “type 1” pattern on the electrocardiogram (ECG) in the right chest leads (V1–V3). NCBI

Brugada syndrome is a heart rhythm condition that makes the lower chambers of the heart (ventricles) prone to dangerous rhythms like ventricular fibrillation. When this happens, the heart cannot pump blood, and a person can faint or die suddenly. In some people, Brugada syndrome is caused by a harmful change (pathogenic variant) in CACNB2, a gene that makes a helper (β2) part of the L-type calcium channel. This defect lowers the calcium current in heart cells and makes the electrical signal across the right ventricle uneven, which can trigger fatal rhythms—especially during fever, certain medicines, or electrolyte problems. NCBI+2PMC+2

What CACNB2 does:

CACNB2 is the β2 subunit that helps the L-type calcium channel open and inactivate properly. When CACNB2 is damaged, the calcium current (ICaL) is reduced (“loss of function”), which can unmask the Brugada ECG pattern and raise the risk of dangerous rhythms. Human cell models from patients with CACNB2-Brugada confirm this loss-of-function mechanism. NCBI+2PMC+2

Some people have Brugada syndrome because of a change (mutation) in the CACNB2 gene. This gene makes the beta-2 subunit of the L-type calcium channel in heart cells. A mutation can weaken calcium current into the cell. That loss of calcium current can tip the electrical balance in the right ventricle and helps create the Brugada ECG pattern and arrhythmias. Human stem-cell–derived heart cells from patients with CACNB2 variants show reduced L-type calcium channel function, supporting this mechanism. PMC+1

Modern guidelines explain how we diagnose Brugada syndrome, how we test for it with sodium-channel blocker drug challenges, and how we try to prevent sudden death. These guidelines also describe how genetics, such as CACNB2 and other channel genes, contribute to the disease. OUP Academic+1

Other names

Brugada syndrome is also known as:

• BrS

• Idiopathic ventricular fibrillation with coved ST elevation in V1–V3

• Channelopathy with right precordial ST elevation
  These terms all point to the same clinical entity first defined by the Brugada brothers, with diagnosis anchored in the type-1 ECG pattern and risk of ventricular arrhythmia. NCBI

Types

1. ECG pattern types.
   Doctors refer to type 1 (coved ST elevation ≥2 mm with negative T wave in V1–V2) as the diagnostic pattern. Types 2 and 3 show a “saddleback” shape and are not diagnostic by themselves; they may need a drug challenge or higher lead placement to reveal type 1. OUP Academic
2. Spontaneous vs. provoked type 1.
   Some people show type 1 on a routine ECG (spontaneous). Others only show it after a sodium-channel blocker test (for example, ajmaline, flecainide, or procainamide), which provokes the pattern by reducing sodium current and unmasking the substrate. Spontaneous type 1 usually carries higher risk. OUP Academic+1
3. Inherited Brugada vs. phenocopy.
   True Brugada is a channelopathy (often genetic). Sometimes fever, electrolyte problems, or certain drugs can mimic the ECG pattern; this is called a Brugada phenocopy. Treating the cause can resolve the pattern. OUP Academic
4. Genotype-positive vs. genotype-negative.
   About 20–30% of cases have a known pathogenic variant (most often SCN5A; rarer in calcium-channel genes like CACNB2). Many patients have no identified mutation but still meet clinical criteria. Genotype can inform family screening and may influence testing and management. Nature

Causes

Each item below explains a simple mechanism or context. “Primary” means the disease itself; “secondary/phenocopy” means outside triggers that can reveal or imitate it.

1. CACNB2 mutation (primary).
   Loss-of-function in the calcium channel beta-2 subunit weakens inward calcium current (ICa,L), shifting currents in the right ventricular outflow tract, favoring the type-1 pattern and arrhythmias. PMC+1

2. SCN5A mutation (primary).
   Loss of cardiac sodium current (INa) reduces conduction reserve and accentuates early repolarization, the classic and most common genetic cause. Nature

3. Other calcium channel genes (primary).
   Variants in CACNA1C or CACNA2D1 can also reduce ICa,L and promote the Brugada substrate. Nature

4. Potassium channel and related genes (primary).
   Changes that increase outward currents (e.g., KCND3) can deepen the notch in the action potential and favor the coved ST elevation. Nature

5. Compound or multiple variant effects (primary).
   Two or more modest variants in ion-channel or channel-interacting genes can act together to lower the safety margin for conduction. Nature

6. Fever (trigger/phenocopy).
   High temperature can reduce sodium current and unmask the Brugada pattern or trigger arrhythmias; fever control is important. OUP Academic

7. Sodium-channel–blocking drugs (provocation/phenocopy).
   Class I antiarrhythmics (ajmaline, flecainide, procainamide, pilsicainide) reduce INa and can reveal the ECG pattern used for diagnosis; other unintended drugs with sodium-blocking properties can induce a phenocopy. OUP Academic+1

8. Psychotropic medications (phenocopy).
   Some antidepressants and antipsychotics have sodium-blocking effects and may provoke Brugada-like ECG changes or arrhythmias in susceptible hearts. OUP Academic

9. Cocaine and other sodium-blocking substances (phenocopy).
   Illicit or local anesthetic exposure can reduce INa and unmask the pattern. OUP Academic

10. Electrolyte imbalance (phenocopy).
    Low potassium, low sodium, or calcium shifts can change action-potential balance and reveal a coved ST segment elevation. OUP Academic

11. Large meals or alcohol (trigger).
    Vagal surges after heavy meals or at night may promote arrhythmias in Brugada by slowing conduction further. OUP Academic

12. Autonomic changes during sleep (trigger).
    Night-time increased vagal tone is a common setting for events, explaining many nocturnal episodes. NCBI

13. Right ventricular outflow tract structural heterogeneity (modifier).
    Subtle structural or conduction differences in this region can amplify the electrical imbalance in Brugada. OUP Academic

14. Ischemia or coronary spasm (phenocopy).
    Transient ischemia can mimic Brugada patterns; fixing the ischemia normalizes the ECG. OUP Academic

15. Hyperthermia from infection (trigger).
    Even mild infections causing fever may unmask ECG changes; antipyretic treatment is recommended. OUP Academic

16. Thyroid or metabolic derangements (modifier/phenocopy).
    Thyroid extremes and acid-base shifts alter channel function and can accentuate the pattern. OUP Academic

17. Postpartum or hormonal shifts (modifier).
    Hormonal changes may affect ion currents; Brugada is more common and more severe in males, possibly linked to testosterone effects on currents. NCBI

18. High intercostal ECG lead placement reveals latent pattern (diagnostic unmasking).
    Moving V1–V2 up one or two spaces increases sensitivity by better aligning with the right ventricular outflow tract. OUP Academic

19. Genetic background without a single dominant mutation (polygenic).
    Many people lack a single causative variant; cumulative small-effect variants can still produce the phenotype. Nature

20. Age-related changes in conduction (modifier).
    With age, conduction reserve can narrow, making it easier for triggers to tip the balance toward a Brugada phenotype. NCBI

Symptoms

1. No symptoms (common).
   Many people feel completely well. Brugada is often found on a routine ECG or during family screening. The risk still exists even without symptoms. NCBI

2. Fainting (syncope).
   A sudden blackout can occur because the heart beats too fast and cannot pump blood to the brain. Episodes often happen at night or at rest. NCBI

3. Near-fainting or dizziness.
   Lightheaded spells can reflect short runs of abnormal rhythm that stop on their own. NCBI

4. Palpitations.
   A feeling that the heart is racing or pounding can signal ventricular arrhythmia or frequent extra beats. NCBI

5. Nocturnal breathing pauses or gasping noted by others.
   This can happen during an arrhythmic event in sleep; observers may report abnormal breathing or snoring. NCBI

6. Seizure-like activity during a faint.
   Brief shaking can happen from lack of brain blood flow during a cardiac event (not epilepsy). NCBI

7. Sudden cardiac arrest, resuscitated.
   Some first presentations are life-threatening rhythms requiring CPR and defibrillation. NCBI

8. Chest discomfort without blocked arteries.
   Arrhythmias or autonomic swings can cause chest sensations even when coronary arteries are normal. OUP Academic

9. Symptoms with fever.
   During fever, patients may notice more palpitations, presyncope, or syncope; fever control can reduce risk. OUP Academic

10. Symptoms after alcohol or heavy meal.
    Post-meal vagal shifts can trigger arrhythmia in susceptible individuals. OUP Academic

11. Worsening at night or during rest.
    Events commonly cluster during sleep due to autonomic changes. NCBI

12. Family history of sudden death or fainting.
    Relatives may have had unexplained sudden death under age 45, especially males. NCBI

13. Exercise is usually not the trigger.
    Unlike some other conditions, events in Brugada more often occur at rest, not peak exertion. NCBI

14. Anxiety or fear around sleep.
    People who have had night events may fear sleeping; counseling and safety planning can help. NCBI

15. Noisy or irregular heartbeat felt in the neck.
    This can be a sign of rapid arrhythmia with reduced forward flow. NCBI

Diagnostic tests

Below are the test groups your clinician may use. I explain what each test does and why it matters. I divide them into Physical Exam, Manual (bedside) tests, Lab/Pathological tests, Electrodiagnostic tests, and Imaging tests.

A) Physical Exam

1. Vital signs and fever check.
   Doctors check temperature, pulse, and blood pressure. Fever can unmask Brugada changes and raise arrhythmia risk; quick fever treatment is important. OUP Academic

2. Focused cardiac exam and signs of poor perfusion.
   A normal heart exam does not rule out Brugada. But signs like low blood pressure during a spell may suggest an arrhythmic cause. OUP Academic

3. Neurologic check after syncope.
   Brief shaking can mimic epilepsy. A normal neurologic exam after a faint pushes clinicians to evaluate the heart more closely. NCBI

4. Medication and substance review (part of physical encounter).
   Clinicians screen for drugs that block sodium channels or prolong conduction; stopping these can normalize a phenocopy. OUP Academic

B) Manual (bedside) tests without drugs

5. Right precordial ECG lead repositioning (V1–V2 higher).
   Moving V1–V2 up one or two intercostal spaces makes the ECG more sensitive for the Brugada pattern because it “looks” more directly at the right ventricular outflow tract. OUP Academic

6. Serial ECGs at rest and during fever.
   Repeating ECGs when the patient’s physiology changes can reveal a hidden type-1 pattern; treating fever is part of the test strategy. OUP Academic

7. Provocative autonomic maneuvers (clinically selective).
   Shifts in vagal tone (e.g., during sleep or after meals) may accentuate the pattern; capturing ECGs in those states can be informative though not formally standardized. OUP Academic

8. Family screening ECGs.
   Checking first-degree relatives can uncover silent cases and supports a heritable channelopathy rather than a phenocopy. NCBI

C) Lab and Pathological tests

9. Electrolytes (K, Na, Ca, Mg), renal and acid-base panels.
   Electrolyte shifts can mimic or worsen Brugada ECG; correcting them is necessary before labeling a true Brugada pattern. OUP Academic

10. Fever/infection markers (CBC, CRP) and thyroid tests.
    These help find reversible triggers (infection, thyroid extremes) that can unmask phenocopies. OUP Academic

11. Comprehensive drug/toxin screen when appropriate.
    Cocaine and other sodium-blocking substances can induce a Brugada-like ECG; identifying them guides management. OUP Academic

12. Genetic testing (including CACNB2).
    A gene panel can detect pathogenic variants in sodium, calcium, or potassium channel genes. Finding a CACNB2 mutation supports a calcium-channel mechanism and helps with family counseling. Nature

13. Cascade genetic testing for relatives.
    If a pathogenic variant is found in the proband, testing family members can identify silent carriers who need ECG follow-up and fever precautions. NCBI

D) Electrodiagnostic tests

14. Standard 12-lead ECG with focus on V1–V3.
    The cornerstone test. A spontaneous type 1 pattern makes the diagnosis when clinical criteria are met. OUP Academic

15. Sodium-channel blocker (SCB) drug challenge.
    Ajmaline, flecainide, or procainamide can unmask a diagnostic type-1 pattern in suspected cases with non-diagnostic ECGs. Evidence suggests ajmaline is the most sensitive agent, though all must be given under expert monitoring. OUP Academic+1

16. Signal-averaged ECG (SAECG).
    This specialized ECG looks for “late potentials,” tiny delayed signals that indicate slow conduction and may be present in Brugada. It can aid risk assessment, though not diagnostic alone. OUP Academic

17. Holter or patch monitoring.
    24–72-hour (or longer) ECG monitoring can capture intermittent type-1 pattern, frequent premature beats, or runs of ventricular arrhythmia. OUP Academic

18. Electrophysiology study (EPS).
    An invasive test that maps heart conduction and tries to induce ventricular arrhythmias. It can help with risk stratification in selected patients per guideline discussion. OUP Academic

19. Shanghai Score System (clinical diagnostic score).
    This score weighs ECG (spontaneous or drug-provoked type-1), symptoms, family history, and genetics. It helps standardize diagnosis and reduce over- or under-diagnosis. Tools like MDCalc and original papers describe how to use it. jacc.org+2jacc.org+2

E) Imaging tests

20. Transthoracic echocardiography.
    An ultrasound checks chamber size, pumping function, and valves. Brugada usually has a structurally normal heart; echo helps exclude other causes. OUP Academic

21. Cardiac MRI (CMR).
    CMR looks for scar or inflammation and characterizes the right ventricle and outflow tract. It helps rule out conditions that can mimic Brugada (e.g., myocarditis, arrhythmogenic right ventricular cardiomyopathy). OUP Academic

Non-pharmacological treatments (therapies & “other” measures)

Each item includes a brief description (≈150 words), purpose, and mechanism in simple language.

1) Avoid unsafe medicines (use a trusted list every time)
Description: Some medicines can worsen the Brugada ECG pattern or trigger dangerous rhythms. Before starting any new drug—even antibiotics, allergy pills, or anesthetics—check a Brugada-safe/avoid list made by expert cardiologists. Share the list with your family, pharmacist, dentist, and anesthetist. If an unsafe drug is the only option, doctors may use hospital monitoring.
Purpose: Reduce rhythm triggers from drug-induced sodium or calcium channel effects.
Mechanism: Many “avoid” drugs block the heart’s sodium or calcium channels, exaggerating the voltage difference across the right ventricle and making ventricular fibrillation more likely. brugadadrugs.org+1

2) Treat fever fast (paracetamol/acetaminophen and cooling)
Description: Fever is a powerful trigger in Brugada syndrome. Treat any temperature rise promptly with antipyretics and fluids; seek urgent medical help if you feel faint, dizzy, or notice palpitations during a fever. Families often keep antipyretics at home and a thermometer handy.
Purpose: Prevent fever-induced ECG changes and arrhythmias.
Mechanism: Heat changes the function of cardiac ion channels and can unmask the type-1 Brugada pattern; lowering body temperature reduces this stress on the channels. PMC+1

3) Keep electrolytes in the “normal-to-high-normal” range (especially potassium and magnesium)
Description: Low potassium (hypokalemia) and low magnesium increase ventricular arrhythmia risk in heart patients. Eat potassium-rich foods (unless you have kidney disease) and avoid unnecessary diuretics or laxative overuse. Do not start supplements without clinician advice.
Purpose: Make the heart’s electrical reset phase more stable.
Mechanism: Potassium and magnesium stabilize cardiac action potentials; low levels make ventricular cells easier to trigger into abnormal rhythms. PMC+1

4) Avoid binge alcohol and stimulants (including cocaine and some “energy” products)
Description: Binge drinking, recreational stimulants, and some high-dose caffeine products can provoke arrhythmias and may appear on “avoid” lists for Brugada. If you drink alcohol, do so moderately and never binge.
Purpose: Reduce adrenergic surges and ion-channel interference.
Mechanism: Alcohol and stimulants alter autonomic tone and can block or modulate cardiac ion channels, tilting the heart toward dangerous rhythms in Brugada. brugadadrugs.org

5) Make an emergency plan (family training + local EMS awareness)
Description: Create a plan: who calls emergency services, where the nearest AED is, and which hospital knows inherited arrhythmias. Consider telling your workplace or school. Carry printed instructions (fever plan, medicine list, contacts).
Purpose: Fast response saves brain and life in cardiac arrest.
Mechanism: Early defibrillation breaks ventricular fibrillation; organized response reduces treatment delay. (Plan recommendations appear in Brugada management reviews and guidelines.) PubMed

6) Wear a medical alert ID
Description: A bracelet or wallet card stating “Brugada syndrome—avoid sodium-channel–blocking drugs; treat fever urgently” helps in emergencies (ER, anesthesia, dental care).
Purpose: Prevent unsafe medications and speed correct care.
Mechanism: Alerts clinicians before they prescribe or sedate; reduces exposure to provoking drugs. brugadadrugs.org

7) Genetic counseling and family screening
Description: First-degree relatives should be offered ECGs and, when appropriate, genetic testing, because Brugada can run in families. Counseling explains inheritance, test limits, and implications.
Purpose: Find at-risk family members early and guide prevention.
Mechanism: Identifying carriers allows trigger avoidance, fever plans, and risk-based follow-up before a first event. NCBI

8) Peri-operative/anesthesia planning
Description: Tell surgeons and anesthetists you have Brugada syndrome. Anesthesia teams can avoid risky agents and maintain normal temperature and electrolytes. Peri-operative ECG monitoring is often used.
Purpose: Prevent anesthesia-related arrhythmia triggers.
Mechanism: Careful drug choice, normothermia, and electrolyte control reduce channel stress in CACNB2-Brugada. brugadadrugs.org+1

9) Hospital monitoring during high-risk illnesses
Description: For severe infections, high fevers, or when you must take a necessary “caution” medicine, short-term hospital telemetry may be recommended.
Purpose: Catch and treat arrhythmias immediately.
Mechanism: Continuous ECG detects early ventricular ectopy or storms; rapid care prevents deterioration. PubMed

10) Individualized sports advice (usually OK with precautions)
Description: Most people with Brugada can exercise with basic precautions: avoid training when febrile or dehydrated; warm up and cool down; and stop for palpitations, dizziness, or fainting. Competitive sports decisions are individualized.
Purpose: Keep fitness benefits while minimizing triggers.
Mechanism: Hydration, electrolyte balance, and avoiding fever reduce arrhythmia risk during exertion. PubMed

11) Home temperature control during illness
Description: Use antipyretics, cool fluids, and light clothing; avoid saunas/hot tubs during febrile periods.
Purpose: Lower the chance that fever unmasks the ECG pattern.
Mechanism: Reduces temperature-dependent ion-channel dysfunction in Brugada. PMC

12) Regular follow-up in an inherited arrhythmia clinic
Description: See a cardiologist/electrophysiologist who treats Brugada. Review any fainting, palpitations, shocks (if ICD), and all new medicines.
Purpose: Ongoing risk assessment and plan updates.
Mechanism: Specialist care applies evolving evidence (e.g., ablation candidacy, ICD settings). PubMed

13) Keep vaccinations up to date
Description: Preventing high-fever infections (e.g., influenza) can reduce arrhythmia triggers. Follow national vaccine schedules unless contraindicated.
Purpose: Fewer febrile illnesses = fewer Brugada triggers.
Mechanism: Vaccines lower infection and fever rates that destabilize cardiac ion channels. PubMed

14) Avoid crash diets and severe dehydration
Description: Rapid weight-loss schemes, extreme fasting, vomiting, or diarrheal illness can lower potassium and magnesium and increase arrhythmia risk. Rehydrate and seek care for persistent GI losses.
Purpose: Preserve electrolytes and volume.
Mechanism: Maintains stable action potentials and autonomic balance. PMC+1

15) Educate your pharmacist and primary-care team
Description: Put “Brugada—check BrugadaDrugs.org before new prescriptions” in your chart. Ask your pharmacist to screen for interactions.
Purpose: Create safety checks at every prescribing step.
Mechanism: Systematic drug vetting reduces exposure to provoking agents. brugadadrugs.org

16) Night-time illness plan
Description: Because events can occur at rest or during sleep, families agree on a “fever at night” routine: antipyretic dose, fluids, and when to go to the ER (e.g., fainting, chest pain, palpitations).
Purpose: Shorten dangerous delays.
Mechanism: Rapid fever control and early evaluation reduce arrhythmic risk. PMC

17) Consider AED access in high-risk households
Description: Some families, especially with prior arrests or multiple affected members, choose a home AED and practice using it. This is individualized and does not replace medical care.
Purpose: Immediate defibrillation if cardiac arrest occurs at home.
Mechanism: Early shock restores rhythm in ventricular fibrillation. (Supported as a public-health measure; individual use is clinician-directed.) PubMed

18) Clear instructions for dentists and procedural sedation
Description: Give your dentist/anesthetist the drug-avoidance list and fever plan. Schedule procedures when you are well.
Purpose: Prevent sedation-related triggers.
Mechanism: Avoids channel-blocking agents and maintains normothermia. brugadadrugs.org

19) Family ECGs during febrile childhood illnesses (case-by-case)
Description: In families with known Brugada, some clinicians obtain an ECG during significant fevers in children who are genotype-positive or symptomatic.
Purpose: Early detection of a type-1 pattern and timely care.
Mechanism: Fever unmasks patterns; ECG shows risk signals. NCBI

20) Keep a written “Brugada care sheet”
Description: A one-page sheet lists your diagnosis, gene (CACNB2), clinic contacts, avoid-drug URL, fever plan, and ICD/ablation status. Keep copies at home and work.
Purpose: Ensure correct, fast decisions anywhere.
Mechanism: Reduces errors and delays when seconds matter. brugadadrugs.org+1

Drug treatments

Important honesty: There are no drugs FDA-approved specifically for “Brugada syndrome.” Medicines below are used off-label for Brugada (e.g., stopping acute electrical storms) or to treat related arrhythmias. I cite each drug’s FDA label for what the drug is and how it works, and I cite Brugada/ESC guidance for its off-label use context. Always use with an electrophysiologist’s guidance.

1) Quinidine (Class Ia antiarrhythmic)
Description (≈150 words): Quinidine can reduce recurrent ventricular arrhythmias in Brugada by blocking Ito (transient outward potassium current) and sodium channels, which evens out electrical differences in the right ventricle. Clinicians often try quinidine for recurrent syncope, ICD shocks, or when ablation or ICD are not possible. It requires careful dosing and monitoring for QT prolongation, diarrhea, and drug interactions.
Class: Class Ia antiarrhythmic.
Dosage/Time: Extended-release oral tablets; dose individualized; monitor ECG and levels.
Purpose: Prevent ventricular fibrillation recurrence and suppress premature beats.
Mechanism: Ito and Na+ channel block reduces ST-segment elevation substrate in Brugada.
Side effects: GI upset, thrombocytopenia, QT prolongation, torsades risk, interactions. FDA Access Data+1

2) Isoproterenol (ISUPREL) for acute electrical storm
Description: In ICU settings, continuous IV isoproterenol can stop recurrent ventricular fibrillation during Brugada “storms,” especially when triggered by fever. It increases heart rate and β-adrenergic tone, which boosts calcium current and stabilizes the ECG. This is short-term bridge therapy to ablation/ICD or until fever resolves.
Class: β-adrenergic agonist.
Dosage/Time: IV infusion, titrated to suppress arrhythmias and normalize ST elevation.
Purpose: Abort VF storms acutely.
Mechanism: β-stimulation increases ICaL and reduces phase-2 reentry.
Side effects: Tachycardia, hypotension, arrhythmias; ICU monitoring required. FDA Access Data+1

3) Lidocaine (for acute ventricular arrhythmias if needed)
Description: IV lidocaine is sometimes used for ventricular arrhythmias. In Brugada, its effects vary; it is not routine chronic therapy, but may be used as bridge therapy under monitoring.
Class: Class Ib antiarrhythmic/local anesthetic.
Dosage/Time: IV bolus/infusion per label; adjust by weight and response.
Purpose: Short-term suppression of ventricular ectopy/VT.
Mechanism: Fast Na+ channel block in depolarized tissue.
Side effects: CNS effects, hypotension, bradyarrhythmias; dose carefully. FDA Access Data

4) Procainamide (selected acute situations; caution)
Description: IV procainamide can treat ventricular arrhythmias, but it can also unmask type-1 Brugada ECG, so its use is specialist-directed and situation-specific.
Class: Class Ia antiarrhythmic.
Dosage/Time: IV infusion with telemetry; serum levels monitored.
Purpose: Acute rhythm control when judged appropriate.
Mechanism: Blocks Na+ channels and prolongs repolarization.
Side effects: Hypotension, QRS widening, lupus-like syndrome with chronic use. FDA Access Data

5) Amiodarone (selected scenarios; not first-line for Brugada)
Description: Amiodarone treats life-threatening VT/VF in many settings. In Brugada, it is not a reliable preventive agent; may be considered in complex cases or alongside other measures.
Class: Class III antiarrhythmic with multi-channel effects.
Dosage/Time: IV for acute VT/VF per label; oral transition in non-Brugada indications.
Purpose: Rescue therapy for unstable ventricular arrhythmias.
Mechanism: Multichannel block prolongs refractoriness.
Side effects: Hypotension (IV), thyroid, liver, lung toxicity (long-term). FDA Access Data+1

6) Antipyretics (e.g., acetaminophen/paracetamol) during fever
Description: Not an antiarrhythmic, but crucial “drug therapy” in Brugada. At first sign of fever, take antipyretics and fluids, and seek care if unwell.
Class: Antipyretic/analgesic.
Dosage/Time: Per label dosing; avoid overdosing.
Purpose: Reduce fever-triggered arrhythmias.
Mechanism: Lowers body temperature, stabilizing ion-channel behavior.
Side effects: Liver toxicity in overdose—follow label strictly. PMC

7) Electrolyte repletion (potassium, magnesium) when low
Description: In hospital or guided outpatient care, correcting low K+ or Mg2+ reduces arrhythmia risk. Do not self-supplement high doses.
Class: Electrolyte therapy.
Dosage/Time: Individualized; IV or oral with labs/ECG.
Purpose: Stabilize cardiac conduction.
Mechanism: Restores normal action potential and avoids triggered activity.
Side effects: Hyperkalemia or hypermagnesemia if misused—medical supervision is essential. PMC+1

8) Short-acting β-agonist alternatives when isoproterenol unavailable (specialist use)
Description: In rare settings where isoproterenol isn’t available, other β-agonists may be considered to raise ICaL acutely; this is specialist ICU care only.
Class: β-adrenergic agonists.
Dosage/Time: ICU titration.
Purpose: Break VF storm while arranging ablation/definitive care.
Mechanism: Increases calcium current and heart rate.
Side effects: Arrhythmias, hypotension, ischemia risk—requires monitoring. PubMed

9) Sedatives/analgesics chosen to be Brugada-safe during procedures
Description: Drug selection for pain or sedation should follow the Brugada “safe list.”
Class: Varies.
Dosage/Time: Per procedure.
Purpose: Avoid proarrhythmic agents.
Mechanism: Choose agents without sodium/calcium channel-blocking risk.
Side effects: Procedure-specific. brugadadrugs.org

10) Quinidine (alternative salts/formulations per label availability)
Description: Where gluconate or sulfate formulations exist, clinicians tailor formulation and dose; principles and cautions match item #1.
Class/Dose/Mechanism/SE: As above. FDA Access Data

11–20) Other antiarrhythmics
Evidence does not support routine use of many other antiarrhythmics in Brugada, and some can be harmful. Long-term drug prevention is limited; catheter ablation and ICD remain the mainstays for events/recurrences. (Your electrophysiologist individualizes therapy.) PubMed+1

Dietary molecular supplements

There is no supplement proven to treat or prevent Brugada syndrome. Supplements below support general heart or nerve-muscle health. Use only with clinician approval—some interact with medicines or affect rhythm.

1) Omega-3 (EPA/DHA)
Description (≈150 words): Omega-3 fats from fish support general heart health. Food sources are preferred; supplement benefits for arrhythmias are mixed. If you already have heart disease, some groups suggest about 1 g/day EPA+DHA from food (or supervised supplements).
Dosage: Food-first; discuss any supplement dose with your clinician.
Function/Mechanism: May reduce inflammation and modulate cell membranes that influence ion channels; evidence in arrhythmia prevention is inconsistent. ods.od.nih.gov+1

2) Magnesium
Description: Helps more than 300 enzymes and stabilizes nerve and muscle function, including heart rhythm. Correcting low magnesium can reduce ectopy; high doses can be dangerous.
Dosage: Meet the RDA from food; supplements only if low and supervised.
Function/Mechanism: Supports electrical stability of cardiac cells. ods.od.nih.gov+1

3) Coenzyme Q10
Description: A mitochondrial cofactor and antioxidant. Evidence does not show benefit for Brugada, but it’s studied broadly for heart support; not FDA-approved for any disease.
Dosage: Varies widely; discuss with clinician because of drug interactions (e.g., warfarin).
Function/Mechanism: Antioxidant and mitochondrial electron transport. NCBI+1

4) Potassium (dietary, not routine pills)
Description: Emphasize potassium-rich foods (bananas, leafy greens, beans) unless you have kidney disease. Supplements are not routine and can be dangerous.
Dosage: Food-based intake per national guidance; avoid self-supplement pills unless prescribed.
Function/Mechanism: Supports normal cardiac repolarization; low K+ raises arrhythmia risk. PMC

5) Vitamin D
Description: Important for overall health; no evidence it affects Brugada risk.
Dosage: Correct deficiency per clinician advice.
Function/Mechanism: Hormone/vitamin with wide cellular effects; not a rhythm drug. PubMed

6) Riboflavin (B2) and general B-complex from diet
Description: Supports energy metabolism; no Brugada-specific benefit shown.
Dosage: Meet needs via diet; supplements only for deficiency.
Function/Mechanism: Coenzymes in energy pathways. PubMed

7) Taurine (food-sourced amino sulfonic acid)
Description: Sometimes discussed for membranes and calcium handling; not proven for Brugada.
Dosage: Food-based; avoid high-dose supplements without supervision.
Function/Mechanism: Modulates ion fluxes in experimental settings. PubMed

8) L-Carnitine
Description: Fatty-acid transport cofactor; no Brugada evidence.
Dosage: Diet first; supplement only for specific deficiencies.
Function/Mechanism: Mitochondrial energy shuttling. PubMed

9) Selenium (adequacy only)
Description: Trace element; severe deficiency affects the heart, but supplementation beyond adequacy is not helpful and can be toxic.
Dosage: Meet RDA; avoid excess.
Function/Mechanism: Antioxidant enzymes. PubMed

10) Zinc (adequacy only)
Description: Immune and enzyme support; no role in Brugada treatment.
Dosage: Meet needs; avoid high-dose pills.
Function/Mechanism: Numerous enzyme systems; not antiarrhythmic. PubMed

Immunity booster / regenerative / stem-cell drugs

Reality check: There are no approved “immunity boosters,” regenerative medicines, or stem-cell drugs that treat or prevent Brugada syndrome. If you see such claims, they are not evidence-based. Safer options are: fever prevention (vaccines), rapid fever treatment, guideline-based arrhythmia care, and carefully selected ablation/ICD when indicated. Ask your specialist about clinical trials if interested. PubMed+1

(For completeness, clinicians sometimes use left cardiac sympathetic denervation for other inherited arrhythmias with adrenergic triggers; it’s not standard for Brugada and is considered case-by-case by expert teams.) PMC

Surgeries/procedures (what they are & why done)

1) Implantable cardioverter-defibrillator (ICD)
Procedure/Why: A small device placed under the skin with leads to the heart (or under the skin only with S-ICD) that detects and shocks life-threatening rhythms. It is the most effective life-saving therapy for survivors of cardiac arrest or high-risk Brugada patients after specialist risk assessment. PubMed+1

2) Subcutaneous ICD (S-ICD)
Procedure/Why: An ICD with a lead under the skin instead of inside veins. Avoids some lead-related complications in younger patients; choice depends on pacing needs and anatomy. sads.org

3) Epicardial substrate catheter ablation (right ventricular outflow tract/anteroseptal RV epicardium)
Procedure/Why: An electrophysiologist maps and ablates the abnormal electrical substrate, often on the RV epicardium, to prevent VF recurrences and reduce ICD shocks. Effective in selected symptomatic Brugada patients. PMC+1

4) Combined endocardial–epicardial ablation (complex cases)
Procedure/Why: When mapping shows broader substrate, both surfaces are targeted to eliminate triggers and substrate, usually after recurrent storms or shocks. PMC

5) Left cardiac sympathetic denervation (LCSD) in exceptional cases
Procedure/Why: Minimally invasive removal of part of the left sympathetic chain to reduce adrenergic triggers—mainly used for other inherited channelopathies; occasionally considered by expert centers in refractory arrhythmias. PMC+1

Prevention points (simple and practical)

1. Treat fever immediately and seek care if unwell. PMC

2. Check every new medicine at BrugadaDrugs.org and with your pharmacist. brugadadrugs.org

3. Keep electrolytes healthy (food-based potassium/magnesium; no unsupervised pills). PMC+1

4. Avoid binge alcohol and stimulants/recreational drugs. brugadadrugs.org

5. Stay hydrated during illness and exercise. PubMed

6. Have a written plan (fever, drugs to avoid, emergency contacts). PubMed

7. Tell all clinicians (dentist, surgeon, anesthetist) about Brugada before procedures. brugadadrugs.org

8. Family screening for first-degree relatives. NCBI

9. Regular specialist follow-up (inherited arrhythmia clinic). PubMed

10. Discuss ICD/ablation promptly after any serious event or recurrent arrhythmias. PubMed

When to see a doctor (or go to the ER)

See a doctor now (ER) for fainting, severe dizziness, palpitations, chest pain, or any event during a fever—especially if you already carry a Brugada diagnosis or have CACNB2-Brugada in the family. If you are newly diagnosed, arrange prompt electrophysiology follow-up to discuss risk, safe medications, and whether ablation or ICD is needed. During any surgery or dental procedure, remind the team about Brugada and the drug-avoidance list. PubMed+1

What to eat” and “what to avoid

Eat more of:
• Potassium-rich foods (leafy greens, beans, bananas, oranges, potatoes) if your kidneys are healthy. PMC
• Magnesium-rich foods (nuts, seeds, legumes, whole grains). ods.od.nih.gov
• Fish 1–2×/week for omega-3s (food first). ods.od.nih.gov
• Plenty of fluids during illness/exercise to avoid dehydration. PubMed
• Overall heart-healthy pattern (vegetables, fruits, whole grains, legumes). PubMed

Avoid or limit:
• Binge alcohol and recreational drugs/stimulants. brugadadrugs.org
• High-dose “energy” products with unknown stimulant blends. brugadadrugs.org
• Self-prescribed potassium/magnesium pills (unless your clinician says so). ods.od.nih.gov
• Crash diets/extreme fasting that cause electrolyte loss. PMC
• Any new supplement without checking interactions and safety. NCBI

Frequently asked questions

1) What exactly is CACNB2-Brugada?
A Brugada syndrome subtype where a harmful change in the CACNB2 gene reduces L-type calcium current in heart cells, increasing the risk of dangerous rhythms. PMC+1

2) How is it found?
By a typical ECG pattern (often type-1, sometimes during fever or after a drug challenge) plus clinical history and genetic testing. PubMed

3) Why is fever a problem?
Heat alters ion-channel function and can unmask the Brugada pattern and trigger arrhythmias—so treat fever fast. PMC

4) Are there medicines I must never take?
Yes—some drugs can provoke events. Always check an expert list such as BrugadaDrugs.org before starting any medicine. brugadadrugs.org

5) Can diet cure Brugada?
No. Diet supports general heart health. Brugada treatment focuses on trigger control, ablation, and ICD where needed. PubMed

6) Is there an FDA-approved drug for Brugada?
No. Drugs like quinidine (off-label) and isoproterenol (for storms) are used by specialists; their labels don’t list Brugada as an indication. FDA Access Data+1

7) What about supplements?
No supplement treats Brugada. Avoid unsupervised potassium/magnesium pills and high-dose “heart” supplements. Food first. ods.od.nih.gov+1

8) Will I need an ICD?
If you had cardiac arrest, sustained VT/VF, or are otherwise high-risk after full evaluation, an ICD saves lives. Decisions are individualized. PubMed

9) Can catheter ablation “fix” Brugada?
In selected patients with recurrent VF/shocks, ablation of the RV epicardial substrate can greatly reduce recurrences. It’s not for everyone. ahajournals.org

10) Is pregnancy safe?
With planning and a Brugada-aware team, most pregnancies are uneventful. Avoid fever and unsafe drugs; maintain electrolytes. PubMed

11) Can children be affected?
Yes. Families should discuss ECG/genetic screening for first-degree relatives and fever plans for kids. NCBI

12) Can I exercise?
Usually yes, with precautions: no exercise during fever/dehydration; stop for symptoms; get individualized advice. PubMed

13) What should I carry with me?
Medical alert ID, care sheet (diagnosis, gene, avoid-drug link), and emergency contacts. brugadadrugs.org

14) I fainted once years ago—does it matter?
Yes—tell your specialist. Syncope can signal arrhythmic risk and may change management. PubMed

15) What’s the most important daily habit?
Check every new medicine against an expert list and treat fever fast. Those two steps prevent many emergencies. brugadadrugs.org+1

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: November 04, 2025.