Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

INITIAL

with acute ventricular arrhythmia

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isoprenaline

Intravenous isoprenaline should be given during an acute ventricular arrhythmia, including electrical storm (>2 episodes of ventricular tachycardia or ventricular fibrillation in 24 hours) if the mechanism is due to short-coupled premature ventricular complex-induced ventricular fibrillation.[1][7]​​[18]​​[51]

Primary options

isoprenaline: consult specialist for guidance on dose

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Consider – 

quinidine or phosphodiesterase-III inhibitor

Additional treatment recommended for SOME patients in selected patient group

Quinidine may be considered in addition to isoprenaline for patients who experience electrical storm, according to US guidelines.[1][5][51]​​[81]​​​ In practice, this is given once they have been stabilised with isoprenaline.

Phosphodiesterase-III inhibitors (cilostazol or milrinone) may be considered for patients with BrS (as an alternative to quinidine), however, supportive data are limited.[1][7]

Primary options

quinidine sulfate: consult specialist for guidance on dose

Secondary options

cilostazol: consult specialist for guidance on dose

OR

milrinone: consult specialist for guidance on dose

ACUTE

asymptomatic

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conservative management

Conservative management includes:[1][7]

  • Avoidance of drugs that could exacerbate the Brugada pattern (for further information see: BrugadaDrugs.org: safe drug use and the Brugada syndrome Opens in new window).[18][51]​​ These include anti-arrhythmics (particularly sodium-channel blockers), psychotropic drugs, anaesthetics, certain over-the-counter drugs (e.g., antihistamines), illicit drugs (e.g., cannabis, cocaine)

  • Prompt treatment of fever[18][51]

  • Avoidance of metabolic disturbance (e.g., hypokalaemia, hyperkalaemia, metabolic acidosis)

  • Avoidance of alcohol intoxication.[18][51]

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Plus – 

genetic counselling

Treatment recommended for ALL patients in selected patient group

Arrange genetic counselling for all patients to facilitate screening of relatives.​[1][7][18][80]​​ See Screening.​​

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Consider – 

implantable cardioverter defibrillator (ICD)

Additional treatment recommended for SOME patients in selected patient group

An ICD may be considered in asymptomatic patients if they have high-risk features that are considered relevant after consultation with an expert and who:

  • Have a type 1 Brugada pattern on ECG[1][7]

  • Experience one or more episodes of documented spontaneous sustained ventricular tachycardia​​[7][18][51]​​[68]​​​[91]

  • Develop ventricular fibrillation during electrophysiological study with programmed ventricular stimulation.​[7][51]

The patient’s life expectancy should also be taken into account; ICD may not be appropriate if meaningful survival is <1 year.[18]

In general, ICDs should be avoided in patients without high risk features.[1][51]

Back
Consider – 

quinidine or phosphodiesterase-III inhibitor

Additional treatment recommended for SOME patients in selected patient group

Quinidine is a class 1a anti-arrhythmic agent that has many effects.[1] The most significant of these is inhibition of the cardiac transient outward potassium current, which prolongs the refractory period.[1]

Quinidine is useful to suppress ventricular arrhythmias.[1][51] It should be considered in asymptomatic patients who:[1][5]

  • Have an ICD and experience recurrent shocks​[7][18]

  • Decline or are unsuitable for an ICD​[7][18][51]​​​

  • Have a spontaneous type I Brugada pattern on ECG[51]

  • Experience asymptomatic ventricular arrhythmia.[68]

Monitor the patient's FBC for anaemia and thrombocytopenia.[1]

Be aware that significant adverse effects and difficulty in obtaining the drug can limit its use.[1][5]

Phosphodiesterase-III inhibitors (cilostazol or milrinone) may be considered for patients with BrS (as an alternative to quinidine), however, supportive data are limited.[1][7]

Primary options

quinidine sulfate: consult specialist for guidance on dose

Secondary options

cilostazol: consult specialist for guidance on dose

OR

milrinone: consult specialist for guidance on dose

symptomatic

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conservative management

Conservative management includes:[1][7]

  • Avoidance of drugs that could exacerbate the Brugada pattern (for further information see:  BrugadaDrugs.org: safe drug use and the Brugada syndrome Opens in new window).[18][51]​​ These include anti-arrhythmics (particularly sodium-channel blockers), psychotropic drugs, anaesthetics, certain over-the-counter drugs (e.g., antihistamines), illicit drugs (e.g., cannabis, cocaine)

  • Prompt treatment of fever[18][51]

  • Avoidance of metabolic disturbance (e.g., hypokalaemia, hyperkalaemia, metabolic acidosis)

  • Avoidance of alcohol intoxication.[18][51]

Back
Plus – 

genetic counselling

Treatment recommended for ALL patients in selected patient group

Arrange genetic counselling for all patients to facilitate screening of relatives.[1][7][18]​​​[80]​​ See Screening.

Back
Plus – 

implantable cardioverter defibrillator (ICD)

Treatment recommended for ALL patients in selected patient group

An ICD is recommended for all patients with BrS who have been resuscitated from cardiac arrest for secondary prevention of sudden cardiac death.[1][7][18][51]​​[91]

  • The patient’s life expectancy should also be taken into account; ICD may not be appropriate if meaningful survival is <1 year.[18]

Regarding choice of ICD, a dual chamber system is recommended in most patients, particularly those prone to atrial arrhythmias (e.g., those carrying the SN5CA gene).[1] However, a subcutaneous device may be preferred in young patients who don’t require pacing, because this has a lower risk of intravascular infection.[1]

Back
Consider – 

quinidine or phosphodiesterase-III inhibitor

Additional treatment recommended for SOME patients in selected patient group

Quinidine is a class 1a anti-arrhythmic agent that has many effects.[1]​ The most significant of these is inhibition of the cardiac transient outward potassium current, which prolongs the refractory period.[1]

Quinidine is useful to suppress ventricular arrhythmias, and may also be used for medical management of atrial arrhythmias.[1][51]​ It should be considered in patients who:[1][5]

  • Have an ICD and experience recurrent shocks​[7][18]

  • Decline, or are unsuitable for, an ICD​[7][18][51]

  • Experience electrical storm (>2 episodes of ventricular tachycardia or ventricular fibrillation in 24 hours).[51] See 'Acute ventricular arrhythmia' above.

  • Experience asymptomatic ventricular arrhythmia.[68]

Monitor the patient's FBC for anaemia and thrombocytopenia.[1]

Be aware that significant adverse effects and difficulty in obtaining the drug can limit its use.[1][5]

Phosphodiesterase-III inhibitors (cilostazol or milrinone) may be considered for patients with BrS (as an alternative to quinidine), however, supportive data are limited.[1][7]

Primary options

quinidine sulfate: consult specialist for guidance on dose

Secondary options

cilostazol: consult specialist for guidance on dose

OR

milrinone: consult specialist for guidance on dose

Back
Consider – 

radiofrequency catheter ablation

Additional treatment recommended for SOME patients in selected patient group

Indicated in patients who:

  • Have recurrent ICD shocks, particularly if these are refractory to medical therapy[1]​​[5][7]​​[18]​​​[51]

  • Experience serious arrhythmic events despite optimised medical therapy[1][5]

  • Are intolerant to or would prefer not to have medical therapy, or medical therapy is ineffective[1][5]

  • Have spontaneous type 1 Brugada pattern on ECG and symptomatic ventricular arrhythmias who are unsuitable for, or decline an ICD[18]

  • Have a history of electrical storms.[51]

Significant contraindications to ablation include:[38]

  • Ventricular tachycardia or fibrillation caused by myocardial ischaemia, fever, or hypokalaemia

  • Presence of structural heart disease

  • Brain anoxic encephalopathy from cardiac arrests caused by ventricular tachycardia or fibrillation.

A combined endocardial and epicardial approach has been shown to modify triggers and substrate for ventricular arrhythmias.[1]

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1st line – 

conservative management

Conservative management includes:[1][7]

  • Avoidance of drugs that could exacerbate the Brugada pattern (for further information see: BrugadaDrugs.org: safe drug use and the Brugada syndrome Opens in new window).[18][51]​​ These include anti-arrhythmics (particularly sodium-channel blockers), psychotropic drugs, anaesthetics, certain over-the-counter drugs (e.g., antihistamines), illicit drugs (e.g., cannabis, cocaine)

  • Prompt treatment of fever[18][51]

  • Avoidance of metabolic disturbance (e.g., hypokalaemia, hyperkalaemia, metabolic acidosis)

  • Avoidance of alcohol intoxication.[18][51]

Back
Plus – 

genetic counselling

Treatment recommended for ALL patients in selected patient group

Arrange genetic counselling for all patients to facilitate screening of relatives.​[1][7][18][80]​​ See Screening.

Back
Consider – 

implantable cardioverter defibrillator (ICD)

Additional treatment recommended for SOME patients in selected patient group

The decision to use an ICD for primary prevention of sudden cardiac death due to BrS is less clear cut than in patients with BrS who have been resuscitated from cardiac arrest, and needs to weigh up the risk of serious arrhythmic events with the risk of ICD-related complications.[1]

An ICD is generally recommended for patients who have:

  • Spontaneous type 1 Brugada pattern on ECG and a history of cardiogenic syncope, particularly if this is likely to be due to ventricular arrhythmias[1][7][18]​​

  • Documented spontaneous sustained ventricular tachycardia with or without syncope.​​[7][18][51]

An ICD should be considered in patients who:

  • Have provoked type 1 Brugada pattern on ECG and a history of cardiogenic syncope[1][7][18]​​

  • Develop ventricular fibrillation during electrophysiological study with programmed ventricular stimulation.[7][51]

The patient’s life expectancy should also be taken into account; ICD may not be appropriate if meaningful survival is <1 year.[18]

Regarding choice of ICD, a dual chamber system is recommended in most patients, particularly those prone to atrial arrhythmias (e.g., those carrying the SN5CA gene).[1] However, a subcutaneous device may be preferred in young patients who don’t require pacing, because this has a lower risk of intravascular infection.[1]

Back
Consider – 

quinidine or phosphodiesterase-III inhibitor

Additional treatment recommended for SOME patients in selected patient group

Quinidine is a class 1a antiarrhythmic agent that has many effects.[1] The most significant of these is inhibition of the cardiac transient outward potassium current, which prolongs the refractory period.[1]

Quinidine is useful to suppress ventricular arrhythmias, and may also be used for medical management of atrial arrhythmias.[1][51]​​ It should be considered in patients who:[1][5]​​

  • Have an ICD and experience recurrent shocks​[7][18]

  • Decline or are unsuitable for an ICD​​[7][18][51]

  • Experience electrical storm (>2 episodes of ventricular tachycardia or ventricular fibrillation in 24 hours)[51] See 'Acute ventricular arrhythmia' above.

  • Experience asymptomatic ventricular arrhythmia.[68]

Monitor the patient's FBC for anaemia and thrombocytopenia.[1]

Be aware that significant adverse effects and difficulty in obtaining the drug can limit its use.[1][5]

Phosphodiesterase-III inhibitors (cilostazol or milrinone) may be considered for patients with BrS (as an alternative to quinidine), however, supportive data are limited.[1][7]

Primary options

quinidine sulfate: consult specialist for guidance on dose

Secondary options

cilostazol: consult specialist for guidance on dose

OR

milrinone: consult specialist for guidance on dose

Back
Consider – 

radiofrequency catheter ablation

Additional treatment recommended for SOME patients in selected patient group

Indicated in patients who:

  • Have recurrent ICD shocks, particularly if these are refractory to medical therapy[1][5][7]​​[18]​​[51]​​​​​

  • Experience serious arrhythmic events despite optimized medical therapy[1][5]

  • Are intolerant to or would prefer not to have medical therapy, or medical therapy is ineffective[1][5]

  • Have spontaneous type 1 Brugada pattern on ECG and symptomatic ventricular arrhythmias who are unsuitable for or decline an ICD[18]

  • Have a history of electrical storms.[51]

Significant contraindications to ablation include:[38]

  • Ventricular tachycardia or fibrillation caused by myocardial ischaemia, fever, or hypokalaemia

  • Presence of structural heart disease.

A combined endocardial and epicardial approach has been shown to modify triggers and substrate for ventricular arrhythmias.[1]

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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