01Approach to the Critical Patient/attcp56

Immediate stabilization

›VF/pulseless VT recognition

›Immediate defibrillation 200 J biphasic

›Standard ACLS protocol

›Repeat shock if VF persists

›CPR quality targets

›Rate 100-120 compressions/min

›Depth 5-6 cm

›Epinephrine 1 mg IV every 3-5 minutes during ACLS

›Amiodarone 300 mg IV for refractory VF

›VF storm management

›IV isoproterenol 1-2 mcg/min continuous infusion

›Titrate to suppress recurrent VF

›Augments L-type calcium current and suppresses Ito

›First-line for electrical storm in BrS, Class I recommendation

›Avoid procainamide and class IC agents

›Worsens sodium channel blockade

›Can precipitate further VF

›Quinidine 200-300 mg PO TID as bridging agent

›Class Ib; blocks Ito current

Monitoring and targets

›Continuous cardiac monitoring

›12-lead ECG on arrival

›Identify type 1 coved pattern in V1-V2

›Check leads V1-V2 placed at 2nd-3rd intercostal space if standard placement non-diagnostic

›Continuous telemetry

›VF or polymorphic VT detection

›Sinus bradycardia increases risk

02History/hx80

Presenting symptoms

›Syncope characterization

›Rest or sleep onset

›Classic BrS pattern; occurs at rest or during sleep

›Not exertional (exertional syncope favors LQTS, CPVT, HCM)

›Prodrome absence

›Often no warning; abrupt loss of consciousness

›Distinguishes from vasovagal syncope

›Nocturnal agonal respirations

›Witnessed by bed partner

›Classic clinical clue for BrS

›Cardiac arrest history

›Prior resuscitated SCA

›Highest risk category: approximately 10-15%/year VF recurrence

›Mandates ICD evaluation

›ICD shock history

›Appropriate versus inappropriate shock determination

›VF storm: ≥3 appropriate shocks within 24 hours

›Palpitations and presyncope

›Palpitations at rest

›May represent self-terminating polymorphic VT

›Seizure-like episodes

›May be misdiagnosed as epilepsy

›Represent self-terminating VF with anoxic seizure

›Chest discomfort

›Atypical; structural disease excluded

Triggers and exposures

›Fever history

›Recent febrile illness

›Fever unmasks or worsens type 1 ECG pattern

›Especially dangerous in children

›Events during or shortly after fever onset

›High clinical suspicion for BrS trigger

›Dietary and substance triggers

›Heavy or large late-evening meals

›Increases vagal tone; postprandial risk period

›Excessive alcohol consumption

›Can precipitate syncope and unmask pattern

›Cocaine use

›Sodium channel blockade; high-risk combination

›Cannabis use

›Associated with arrhythmic events in BrS

›Medication exposures

›Class IA/IC antiarrhythmics

›Flecainide, propafenone, procainamide

›Tricyclic antidepressants

›Amitriptyline, nortriptyline, imipramine, desipramine

›Psychotropics

›Lithium, trifluoperazine, fluoxetine

›Anesthetics

›Propofol, bupivacaine

›Reference brugadadrugs.org for complete list

Family and genetic history

›Family history of SCD

›Unexplained sudden death in relative <45 years

›Present in approximately 26% of BrS patients

›Warrants urgent evaluation of the proband

›Drowning or single-vehicle accidents in relatives

›May represent unrecognized SCD equivalents

›SIDS in infants

›BrS implicated in some pediatric unexplained deaths

›Ethnic background

›Southeast Asian descent

›Higher prevalence; linked to SUNDS (Bangungut, Lai Tai)

›Prevalence estimated at 1 in 2000 worldwide, higher in Southeast Asia

›Inheritance pattern

›Autosomal dominant with variable penetrance

›Greater than 90% of symptomatic patients are male

›Female carriers may be asymptomatic but can transmit

Past medical history

›Prior cardiac events

›Prior syncope episodes especially at rest or sleep

›Prior cardiac arrest or resuscitated SCA

›History of atrial fibrillation

›AF is associated with BrS in subset of patients

›Sinus node dysfunction or AV conduction disease

›Existing devices

›ICD implanted

›Model, date, recent interrogation

›Prior ICD-related complications

›Inappropriate shocks, lead fracture (significant concern given young age)

›Psychiatric history

›Current psychotropic medications

›TCAs, SSRIs, lithium — high-risk list

03Physical Exam/exam42

Vital signs

›Stability assessment

›Temperature

›Fever >38 C is critical modifiable trigger

›Active fever warrants immediate antipyresis

›Heart rate and rhythm

›Bradycardia increases arrhythmic risk

›Sinus pauses and vagotonic states elevate risk

›Blood pressure

›Hypotension suggests hemodynamic compromise or post-event state

›Oxygen saturation

›Desaturation may indicate ongoing resuscitation sequelae

Cardiovascular exam

›Cardiac auscultation

›Typically normal

›BrS patients have structurally normal hearts

›No expected murmurs or gallops

›Irregular rhythm

›Frequent PVCs or AF may be auscultated

›Chest wall inspection

›Pectus excavatum

›Mechanical RVOT compression can produce Brugada-like ECG

›Document and correlate with imaging

›ICD scar or device palpation

›Confirm device position and pocket integrity

Neurologic exam

›Post-ictal or post-arrest state

›Level of consciousness

04Differential Diagnosis/ddx54

Life-threatening mimics

›Acute coronary syndromes

›Acute MI with RV ischemia

›ICD-10 I21

›ST elevation in different distribution; troponin positive

›Differentiating feature

›Troponin negative in BrS

›Type 1 pattern in V1-V2 without lateral ST changes

›Pulmonary embolism

›ICD-10 I26

›Acute RV strain pattern

›DVT risk factors, pleuritic pain, hypoxia

›ECG features

›S1Q3T3 pattern; sinus tachycardia predominant

›Arrhythmogenic right ventricular cardiomyopathy (ARVC)

›ICD-10 I42.8

›Structural RV abnormalities on imaging

›Epsilon waves on ECG

›Key distinction

›ARVC has RV structural changes on echo or MRI

›BrS has structurally normal heart

ECG mimics and Brugada phenocopies

›Brugada phenocopies

›Identifiable underlying condition producing type 1 pattern

›Hyperkalemia: peaked T waves, widened QRS; ICD-10 E87.5

›Hypercalcemia: shortened QT interval; ICD-10 E83.52

›Hypothermia: Osborn J waves; ICD-10 T68

›Pericarditis/myocarditis: diffuse ST changes, PR depression; ICD-10 I30, I40

05Laboratory Tests/lt46

Core metabolic panel

›Basic metabolic panel

›Potassium

›Hyperkalemia unmasks Brugada ECG pattern

›Target K+ 3.5-5.0 mmol/l

›Calcium

›Hypercalcemia can shorten QT and alter precordial pattern

›Hypocalcemia can prolong QT

›Bicarbonate and anion gap

›Metabolic acidosis can unmask BrS pattern

›Correct acidosis to reduce arrhythmic risk

›Creatinine and BUN

›Renal function for drug dosing

›Uremia can affect cardiac conduction

Cardiac biomarkers

›Troponin

›High-sensitivity troponin I or T

›Excludes acute MI as cause of ST elevation

›Expected to be negative in BrS

›Serial troponins if chest pain component

›0 and 3 hour protocol if ACS concern

›BNP or NT-proBNP

›Elevated if cardiomyopathy or sustained VF causes transient dysfunction

›Expected normal in isolated BrS

Toxicology and drug levels

›Urine and serum toxicology

›Cocaine metabolites

›Sodium channel blockade; high-risk combination with BrS

06Diagnostic Tests/dt109

Scoring Systems

›Shanghai Score for Brugada Syndrome diagnosis

›Integrates ECG, clinical, family history, and genetic data

›Score ≥3.5 points: high probability BrS diagnosis

›Score 2-3: intermediate probability; provocative testing indicated

›Score <2: low probability

›ECG components

›Spontaneous type 1: 3.5 points

›Drug-induced type 1: 2 points

›Fever-induced type 1: 2 points

›Clinical components

›Unexplained VF or polymorphic VT: 3 points

›Nocturnal agonal respirations: 2 points

›Syncope suspected arrhythmic: 2 points

›Syncope of unexplained mechanism: 1 point

›Family history component

›First-degree relative with definite BrS: 2 points

›Unexplained SCD <45 in first-degree relative: 1 point

›Genetic testing component

›Pathogenic SCN5A mutation: 0.5 points

›Risk stratification by clinical category

›Prior cardiac arrest: approximately 10-15%/year VF recurrence

›Highest risk; ICD Class I

›Arrhythmic syncope with spontaneous type 1: approximately 2.3%/year event rate

›ICD Class I

›Asymptomatic with spontaneous type 1: approximately 1-9%/year (controversial)

›ICD Class IIa per 2017 AHA/ACC/HRS guidelines

›Asymptomatic with drug-induced type 1 only: <0.4%/year

›Observation; ICD not routinely indicated

MRI

›Cardiac MRI indications in BrS evaluation

›ARVC exclusion

›Fibrofatty replacement of RV myocardium supports ARVC over BrS

›Task Force Criteria for ARVC include MRI-defined RV abnormalities

›RVOT structural assessment

›Emerging evidence of subtle RVOT structural abnormalities in BrS

›Not routinely required for diagnosis

›Myocarditis evaluation

›Late gadolinium enhancement supports inflammatory cause of Brugada phenocopy

›T2 signal elevation in acute myocarditis

›Protocol considerations

›Gadolinium contrast administration

›Required for late gadolinium enhancement assessment

›Contraindicated in severe renal impairment (eGFR <30)

›ICD compatibility

›Conditional MRI-safe ICD required if device already implanted

›Confirm device MRI compatibility before scanning

CT

›CT chest indications

›Mediastinal mass or tumor exclusion

›RVOT compression as Brugada phenocopy

›Lymphoma or other mediastinal mass

›Pulmonary embolism exclusion

›CT pulmonary angiography if PE suspected

›CTPA sensitivity approximately 83%, specificity approximately 96% for PE

›Pectus excavatum assessment

›CT chest defines degree of cardiac compression

›Haller index calculated from CT

›Coronary CT angiography

›ACS exclusion when clinical picture ambiguous

›High-sensitivity troponin first; CCTA for intermediate risk

›Coronary artery anatomy typically normal in BrS

Ultrasound

›Echocardiography

›First-line structural assessment

›Typically normal in BrS; structurally normal heart expected

›LVEF and RV function assessment

›ARVC exclusion

›RV dilatation, wall motion abnormalities, aneurysm

›Fatty infiltration not reliably detected by echo; MRI preferred

›Pericardial effusion

›Myocarditis or pericarditis phenocopy assessment

›RVOT dimensions

›Enlarged RVOT may support ARVC or structural phenocopy

›Point-of-care ultrasound in the ED

›Rapid global function assessment

›Exclude obvious structural cause of ST elevation

›Pericardial effusion detection

›IVC assessment

›Volume status assessment if hemodynamic compromise present

Electrocardiographic studies

›Standard 12-lead ECG

›Type 1 Brugada pattern (diagnostic)

›Coved-type ST elevation ≥2 mm in V1 or V2

›Followed by negative T wave without isoelectric interval

›Highest intercostal lead placement increases sensitivity

›Type 2 pattern (not diagnostic alone)

›Saddle-back ST elevation ≥0.5 mm in V1 or V2

›Requires confirmatory testing

›Sodium channel blocker provocation test

›Indications

›Suspected BrS without spontaneous type 1 pattern

›Asymptomatic relative with family history of BrS or SCD

›Agents

›Ajmaline 1 mg/kg IV over 10 minutes (preferred in Europe)

›Flecainide 2 mg/kg IV over 10 minutes (max 150 mg)

›Procainamide 10 mg/kg IV over 10 minutes (alternative)

›Monitoring requirements

›Continuous 12-lead ECG during and 30 min post-infusion

›Resuscitation equipment immediately available

›Test stopped if type 1 pattern, PVCs, VT, QRS >130% baseline

›Holter monitoring

›Intermittent type 1 pattern detection

›Nocturnal ST changes during vagotonic periods

›Spontaneous PVCs or runs of polymorphic VT

›Ambulatory arrhythmia burden

›AF detection (associated with BrS in subset)

›Programmed ventricular stimulation (PVS)

›Role remains controversial

›Class IIb for asymptomatic patients with spontaneous type 1

›Specificity decreases with triple extrastimuli protocol

›Not routinely recommended for risk stratification in most guidelines

›Performed by electrophysiology

›In-patient procedure under monitoring

07Disposition/dispo48

Immediate admission indications

›ICU or monitored admission required

›Cardiac arrest survivor

›Post-ACLS care

›Targeted temperature management if comatose post-arrest

›Electrophysiology consultation for ICD planning

›VF storm or recurrent ICD shocks

›IV isoproterenol infusion requirement

›EP emergency consultation

›Documented syncope with spontaneous type 1 Brugada ECG

›High arrhythmic syncope risk

›Cardiology consultation within hours

›Fever with new type 1 Brugada ECG pattern

›Monitor until afebrile and ECG pattern resolved

›Antipyresis and active cooling maintained

›Active drug intoxication unmasking Brugada pattern

›TCA overdose or cocaine toxicity management

›Pattern expected to resolve with drug clearance

Telemetry ward admission

›Monitored ward appropriate for

›New incidental type 1 Brugada pattern without symptoms

›Cardiology consultation and risk stratification workup

›Provocation test if drug-induced pattern

›Known BrS patient with non-life-threatening trigger

›Fever without hemodynamic compromise after control

›New medication exposure without event

Discharge criteria

›Asymptomatic with drug-induced-only type 1 ECG

08Treatment/t99

Acute VF management

›Defibrillation

›Immediate unsynchronized cardioversion 200 J biphasic

›First intervention for VF or pulseless VT

›Class I recommendation, AHA ACLS guidelines

›ACLS protocol continuation if VF persists

›Epinephrine 1 mg IV every 3-5 minutes

›Amiodarone 300 mg IV for refractory VF

›Second dose 150 mg IV if needed

›CPR quality optimization throughout

VF storm treatment

›IV isoproterenol

›Mechanism

›Augments L-type calcium current

›Suppresses transient outward potassium current (Ito)

›Restores phase 1 epicardial action potential

›Dosing

›1-2 mcg/min continuous IV infusion

›Titrate to heart rate 90-100 bpm

›Increase to 5 mcg/min if VF recurs despite lower dose

›Contraindications

›Avoid in patients with obstructive HCM or LVOTO

›Use with caution in coronary disease

›Evidence

›Class I recommendation per 2017 AHA/ACC/HRS VT/VF guidelines

›Acute suppression of VF storm; bridge to quinidine or ablation

›Quinidine for VF storm bridging

›Mechanism

›Class IA agent

›Blocks Ito current; restores epicardial action potential dome

›Dosing

›Quinidine gluconate 648 mg PO every 8 hours

›Or quinidine sulfate 200-400 mg PO TID

›Monitoring

›QTc monitoring: withhold if QTc >550 ms

›Adverse effects in approximately 38% of patients (diarrhea, thrombocytopenia)

›Evidence

›Class I for VF storm; Class IIa for symptomatic BrS when ICD not feasible

›No SCD observed in long-term quinidine follow-up studies

Fever management

›Aggressive antipyresis

›Acetaminophen 1 g IV/PO every 6 hours

›First-line; initiate immediately for any fever in BrS patient

›Ibuprofen 400-600 mg PO every 6-8 hours if acetaminophen insufficient

›Alternative if no contraindications

›Active cooling measures

›Cooling blanket or ice packs to axillae and groin

›Target temperature <37.5 C

›Fever monitoring

›Hourly temperature checks until afebrile for 12 hours

Electrolyte and trigger correction

›Potassium repletion

›Target K+ 4.0-5.0 mmol/l in BrS patients

›IV potassium chloride 10-20 mmol/hour for significant deficit

›Oral supplementation for mild deficit

›Calcium correction

›Hypercalcemia correction per standard protocols

›IV fluids plus calcitonin or bisphosphonate as indicated

›Calcium administration if severe hypocalcemia

›Calcium gluconate 1-2 g IV over 10-20 minutes

›Medication discontinuation

›All class IA/IC antiarrhythmics ceased immediately

›All TCAs and implicated psychotropics ceased

›Alternative non-BrS-risk agents substituted in consultation with prescribers

Definitive therapy and device management

›ICD implantation

›Class I indications per 2017 AHA/ACC/HRS guidelines

›Spontaneous type 1 ECG plus cardiac arrest, sustained VT, or arrhythmic syncope

›Class IIa indication

›Asymptomatic with spontaneous type 1 ECG

›Programmed stimulation-inducible VF

›Not indicated

›Asymptomatic with drug-induced type 1 pattern only

›Fever-induced-only pattern in otherwise asymptomatic patient

›ICD complications to counsel

›Inappropriate shocks (most common complication in BrS; young age and long device life)

›Lead fracture and sensing issues over decades of follow-up

›Catheter ablation

›Class I for recurrent VF storms unresponsive to medical therapy

›Epicardial ablation of RVOT substrate

›Eliminates type 1 pattern in >75% of cases

›Markedly reduces VF recurrence in refractory VF storm

›Performed by experienced EP centers

›Epicardial access required

›General anesthesia with hemodynamic monitoring

Medications to avoid

›Sodium channel blocking agents

›Class IA: flecainide, propafenone, procainamide

›Class IC: note: flecainide used diagnostically only in monitored setting

›Mechanism of harm

›Reduces Vmax of sodium channel phase 0

›Exaggerates phase 1 epicardial action potential loss

›Precipitates VF

›Psychotropic agents

›TCAs: amitriptyline, nortriptyline, imipramine, desipramine

›Lithium

›Trifluoperazine, fluoxetine

›Anesthetics

›Propofol infusion (high-dose)

›Mechanism includes sodium channel blockade

›Bupivacaine local anesthetic

›Systemic absorption risk perioperatively

›Complete reference

›brugadadrugs.org maintained by consensus group

›Updated regularly; consult before prescribing any new agent

09Special Populations/sp75

Pregnancy

›Physiologic considerations

›Autonomic changes in pregnancy

›Increased sympathetic tone in late pregnancy may be protective

›Peripartum and postpartum vagal surges can increase risk

›Hormonal effects

›Progesterone and estrogen may reduce sodium channel expression

›Variable effect on BrS phenotype expression during pregnancy

›Risk management in pregnancy

›Continue existing ICD during pregnancy

›Fetal radiation exposure from standard device function is negligible

›Perioperative planning for cesarean delivery

›Fever management

›Aggressive antipyresis with acetaminophen (safe in all trimesters)

›Ibuprofen: avoid after 20 weeks gestation (NSAIDs risk premature ductus closure)

›Anesthetic considerations

›Inform anesthesiologist of BrS diagnosis before any procedure

›Avoid propofol infusion; use alternative anesthetic agents

›Bupivacaine neuraxial preferred over IV administration; epidural safe

›Delivery planning

›Multidisciplinary team: cardiology, maternal-fetal medicine, anesthesia

›Telemetry during labor

›Defibrillator immediately available in delivery suite

›Genetic counseling

›Autosomal dominant: 50% transmission risk to offspring

›Prenatal counseling recommended

›Pediatric ECG screening of newborns of BrS parents

›Initial ECG at birth and repeated in childhood

Geriatric

›Age-related considerations

›BrS predominantly presents in 3rd-4th decade

›Late-presenting older adults may have attenuated phenotype

›Comorbidities more common; complicate management

›Polypharmacy risk

›Increased likelihood of receiving contraindicated medications

›Medication reconciliation essential at every encounter

›Brugadadrugs.org review at each new prescription

›ICD considerations in older adults

›Risk-benefit discussion

›Longer device longevity less of a concern in elderly

›Comorbidities may limit benefit of ICD

›Lead complications

›Vascular access issues with multiple prior lines

›Subcutaneous ICD (S-ICD) may be preferred if pacing not required

›Fever management in elderly BrS patients

›Higher baseline infection risk

›Pneumonia and UTI common fever sources

›Aggressive source identification and treatment

›Acetaminophen dosing

›Reduce dose to 500 mg every 6 hours if hepatic impairment

›Avoid NSAIDs with renal impairment or anticoagulation

Pediatrics

›Epidemiology and risk in children

›Rare but aggressive presentation in children

›Fever-triggered events particularly dangerous

›BrS implicated in unexplained pediatric sudden death and SIDS

›SCN5A mutations more commonly identified in pediatric cases

›Genetic testing yield higher in pediatric cohort

›ECG interpretation in children

›Right precordial ST changes common in healthy children

›Age-adjusted normal ranges required

›Standard adult criteria may not apply

›High-lead placement (V1-V2 in 2nd-3rd ICS) recommended

›Increases sensitivity for type 1 pattern detection

›Treatment in pediatric BrS

›Fever management is most critical intervention

›Acetaminophen 15 mg/kg PO/IV every 4-6 hours (max 5 doses/24 hours)

›Ibuprofen 10 mg/kg PO every 6-8 hours as alternative

›Parents educated on fever response plan before discharge

›ICD in children

›Class I for cardiac arrest survivor

›Class I for symptomatic sustained VT

›Subcutaneous ICD (S-ICD) preferred to avoid long-term transvenous lead complications

›2021 PACES guidelines for pediatric device implantation

›Quinidine use in pediatrics

›Class I for symptomatic pediatric patients when ICD not feasible

›Dosing: 15-60 mg/kg/day divided every 6 hours (adjust by age and weight)

›QTc monitoring required

10Background/b85

Epidemiology

›Prevalence and demographics

›Global prevalence approximately 1 in 2000

›Higher prevalence in Southeast Asia

›Estimated 18-28% of unexplained sudden deaths attributable to BrS

›Sex distribution

›Greater than 90% of symptomatic patients are male

›X-linked modifiers proposed but inheritance is autosomal dominant

›Age of presentation

›Peak in 3rd-4th decade

›Mean age of presentation approximately 35-40 years

›Rare pediatric onset; can present at any age

›Genetic epidemiology

›SCN5A mutation identified in 20-30% of phenotype-positive patients

›Most common gene implicated; encodes Nav1.5 sodium channel alpha subunit

›Over 350 mutations described in SCN5A in BrS

›Ethnic predisposition

›SUNDS (sudden unexplained nocturnal death syndrome) in Southeast Asian males

›Also known as Bangungut (Philippines), Lai Tai (Thailand), Pokkuri (Japan)

›Same genetic basis as BrS

›Mortality data

›Untreated cardiac arrest survivors: approximately 10-15%/year recurrence risk

›With appropriate ICD: 10-year survival of cardiac arrest survivors is high

›ICD inappropriate shock rate significant: approximately 20-30% over follow-up

Pathophysiology

›Ionic mechanism

›Loss of action potential dome in RV epicardium

›Imbalance between inward sodium current (INa) and outward potassium current (Ito)

›Reduced INa (SCN5A loss-of-function) allows Ito to dominate phase 1

›Creates phase 1 notch and dome loss in epicardium but not endocardium

›Transmural voltage gradient

›Epicardial-endocardial dispersion of repolarization

›ST elevation in right precordial leads reflects this gradient

›Re-entry mechanism

›Phase 2 re-entry: epicardial dome re-excites adjacent domed area

›Triggers polymorphic VT and VF

›Anatomic predisposition

›Right ventricular outflow tract (RVOT) epicardium

›Highest density of Ito channels in heart

›Epicardial substrate for ablation targets RVOT

›Subtle RVOT structural abnormalities

›Emerging evidence of fibrous tissue in RVOT epicardium in BrS

›May contribute to conduction slowing and re-entry substrate

›Trigger mechanisms

›Fever

›Temperature-sensitive reduction in INa through altered channel gating

›Increases Ito relative dominance at elevated temperatures

›Vagotonic states

›Sleep, postprandial period, rest increase Ito relative contribution

›Explains nocturnal predominance of events

›Sodium channel blocking drugs

›Further reduce already-impaired INa

›Unmask or dramatically worsen phenotype

›Bradycardia

›Rate-dependent sodium channel availability reduced at slower rates

›Vagal bradycardia increases arrhythmic risk

Therapeutic Considerations

›ICD versus medical therapy decision

›ICD is only proven mortality benefit therapy

›Pharmacologic agents reduce event burden but no mortality data equivalent to ICD

›ICD inappropriate shocks and lead complications are significant long-term burden

›Quinidine role

›Reduces VF burden; considered in patients declining ICD or awaiting implant

›Class I for VF storm; Class IIa for symptomatic BrS without ICD

›No deaths observed in long-term quinidine registry data

›Catheter ablation emerging role

›Curative potential with RVOT epicardial substrate elimination

›Greater than 75% of patients have type 1 pattern elimination post-ablation

›Class I for refractory VF storm; expanding to prevention of appropriate ICD shocks

›Trigger avoidance cornerstone

›Fever response plan

›Every BrS patient and family counseled on immediate antipyretic use

›Written fever action plan provided at discharge

›Medication safety

›brugadadrugs.org consultation for every new prescription

›Medical alert identification recommended

›Lifestyle

›Avoid cocaine, cannabis, excess alcohol

›Avoid large late-evening meals

›Adequate hydration to prevent electrolyte abnormalities

›Genetic testing and family screening

›Cascade screening is high yield

›First-degree relatives offered ECG screening

›Sodium channel blocker challenge if ECG non-diagnostic

›SCN5A testing of mutation-positive proband family

›Predictive value of SCN5A

›Positive mutation confirms genetic basis but does not predict severity

›Negative mutation does not exclude BrS (70-80% have no identified mutation)

11Patient Discharge Instructions/pdi49

copy discharge instructions

›Your diagnosis

›Brugada syndrome: an inherited heart rhythm disorder causing risk of sudden cardiac arrest

›Your heart muscle is structurally normal

›The condition affects the electrical system of your heart

›This is a lifelong condition that is manageable with proper precautions

›Fever action plan

›Fever is your most dangerous trigger

›Take acetaminophen (Tylenol) 1000 mg as soon as you notice any temperature above 37.5 C

›Apply cool cloths or take a cool shower to help bring fever down

›If fever does not respond to acetaminophen within 1-2 hours, go to the emergency department

›Do not delay treatment of fever

›Medication safety

›Many common medications are dangerous for you

›Check every new prescription at brugadadrugs.org before taking

›Tell every doctor, dentist, and pharmacist that you have Brugada syndrome

›Carry a medical alert card or wear a medical ID bracelet

›Never take without medical clearance specific to your condition

›Flecainide, propafenone, procainamide (antiarrhythmic drugs)

›Amitriptyline, nortriptyline, or other tricyclic antidepressants

›Cocaine (always strictly avoid)

›Lifestyle instructions

›Avoid excessive alcohol

›Intoxication can trigger dangerous heart rhythms

›Avoid large meals especially late at night

›Large meals increase vagal tone and can trigger events

›Stay well hydrated

›Dehydration and electrolyte imbalance increase risk

12References/r40

Guidelines and key sources

›2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death

›Al-Khatib SM, Stevenson WG, Ackerman MJ, et al.

›Journal of the American College of Cardiology. 2018

›Primary guideline for BrS risk stratification and ICD indications

›EHRA/HRS/APHRS/LAHRS Expert Consensus Statement on Genetic Testing for Cardiac Diseases

›Wilde AAM, Semsarian C, Marquez MF, et al.

›Heart Rhythm. 2022

›Guidance on genetic testing and cascade family screening

›Brugada Syndrome: JACC State-of-the-Art Review

›Brugada J, Campuzano O, Arbelo E, Sarquella-Brugada G, Brugada R.

›Journal of the American College of Cardiology. 2018

›Comprehensive clinical review including quinidine and ablation evidence

›HRS/EHRA/APHRS Expert Consensus Statement on Inherited Primary Arrhythmia Syndromes

›Priori SG, Wilde AA, Horie M, et al.

›Heart Rhythm. 2013

›Foundational consensus for management of BrS and related channelopathies

›Brugada Syndrome. GeneReviews

›Brugada R, Campuzano O, Sarquella-Brugada G, et al.

›NCBI GeneReviews. 2022

›Genetic basis, cascade screening, and long-term management

›2021 PACES Expert Consensus Statement on Cardiovascular Implantable Electronic Devices in Pediatric Patients

›Shah MJ, Silka MJ, et al.

›Heart Rhythm. 2021

›Pediatric ICD indications including BrS

›2024 HRS Expert Consensus Statement on Arrhythmias in the Athlete

›Lampert R, Chung EH, Ackerman MJ, et al.

›Heart Rhythm. 2024

Evidence & Review