Approach

​Treatment for patients with Brugada syndrome (BrS) consists of conservative management and primary and secondary prevention of serious arrhythmic events.[1] T​his aims to prevent serious arrhythmic events, while avoiding potential complications of any treatments.[1] Potential treatments include implantable cardioverter defibrillator (ICD), pharmacologic therapy, and radiofrequency catheter ablation.[1][7]

Note that the distinction between confirmed and probable BrS is not significant in determining the management; this is guided by the patient’s presentation, particularly whether they are symptomatic or asymptomatic. Most patients who are asymptomatic require conservative management only.[1][18]

[Figure caption and citation for the preceding image starts]: Management summary for Brugada syndromeAdapted from Krahn AD et al. J Am Coll Cardiol EP. 2022 Mar, 8 (3) 386-405; used with permission [Citation ends].com.bmj.content.model.Caption@51c8ee56

Acute ventricular arrhythmia

Intravenous isoproterenol 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][18][51]

Quinidine should also be considered for patients who experience electrical storm.​[1][5][51][80]​​ In practice, this is given once they have been stabilized with isoproterenol.

  • Difficulty in obtaining quinidine can limit its use; phosphodiesterase-III inhibitors (cilostazol or milrinone) may be considered as an alternative, although supportive data are limited.[1][7]

Risk stratification

Risk stratify all patients to identify those who are at increased risk of serious arrhythmic events.[1]​ Risk stratification can aid decision making for the management of Brugada syndrome, particularly when considering an ICD for primary prevention of serious arrhythmic events.[1][64]

There are currently no risk stratification tools that have proved effective in clinical practice.[1] The Sieira risk score has been proposed for predicting sudden death in patients with BrS, but has not been externally validated.[57][81][82]​​[83]​​ However, established features that are associated with the greatest risk of serious arrhythmic events are:[1][84][85][86][87]

  • Resuscitated cardiac arrest

  • History of cardiogenic syncope

  • Spontaneous type 1 Brugada pattern on ECG.

Other features that are associated with increased risk of serious arrhythmic events are:[1]

  • Sudden cardiac death in a young (<35 years) first-degree relative

  • Greater "Brugada burden" on ECG, which includes:

    • Presence of type 1 Brugada pattern ECG in the peripheral leads in addition to the right precordial leads

    • Higher proportion of spontaneous type 1 Brugada pattern on ECGs during follow-up

  • Other ECG markers such as early repolarization pattern and QRS fragmentation.[7][64][88]

Age and sex have limited effect on risk of serious arrhythmic events, although age ≥55 at diagnosis is associated with a more benign prognosis, with no increased mortality compared with the general population.[1]

Consider an electrophysiological study with programmed ventricular stimulation using up to two extrastimuli for further risk stratification in some patients, although this remains controversial.[1]​​[7][18]

  • It should not be used routinely because it is invasive and puts the patient at risk of complications, as well as having issues around reproducibility of results.[1]​ One multicenter pooled analysis showed that in patients with BrS, arrhythmias induced with electrophysiologic studies were associated with a higher future risk of ventricular arrhythmia.[89]

  • However, it may be useful in certain circumstances (e.g., where the decision to use an implantable cardioverter defibrillator for primary prevention is unequivocal).[1][18]

Conservative management

Use conservative management for all patients with definite/probable or suspected BrS, although some patients may need other additional treatments alongside this.[1][7]​ Asymptomatic patients with inducible (particularly drug-induced) Brugada pattern on ECG usually require conservative management only.[1][18]

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:

    • Antiarrhythmics (particularly sodium-channel blockers)

    • Psychotropic drugs

    • Anesthetics

    • 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., hypokalemia, hyperkalemia, metabolic acidosis)

  • Avoidance of alcohol intoxication[18][51]

Implantable cardioverter defibrillator (ICD)

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]​​

However, the decision to use an ICD for primary prevention of sudden cardiac death due to BrS is less clear cut and needs to weigh up the risk of serious arrhythmic events with the risk of ICD-related complications.[1]

  • 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]

  • ICD should be considered in patients who:

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

    • Are asymptomatic and have a type 1 Brugada pattern on ECG, with high-risk features that are considered relevant after consultation with an expert.[1][7]​ In general, ICD should be avoided in those patients without high risk features.[1][51]

    • 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]

Pharmacologic therapy

Quinidine

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

  • Are asymptomatic but have a spontaneous type I Brugada pattern on ECG[51]

  • Experience asymptomatic ventricular arrhythmia.[68]

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

  • Monitor the patient's CBC for anemia and thrombocytopenia.[1]

Phosphodiesterase-III inhibitors

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]

Radiofrequency catheter ablation

Radiofrequency catheter ablation is 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 ischemia, fever, or hypokalemia

  • 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]

Genetic counseling

Arrange genetic counseling for all patients to facilitate screening of relatives.[1][7][18][79]​​​​ See Screening.

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