Brugada syndrome
- Overview
- Theory
- Diagnosis
- Management
- Follow up
- Resources
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
with acute ventricular arrhythmia
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
Primary options
isoprenaline: consult specialist for guidance on dose
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [5]Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7967791 http://www.ncbi.nlm.nih.gov/pubmed/31075787?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com [81]Eifling M, Razavi M, Massumi A. The evaluation and management of electrical storm. Tex Heart Inst J. 2011;38(2):111-21. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3066819 http://www.ncbi.nlm.nih.gov/pubmed/21494516?tool=bestpractice.com 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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com
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
asymptomatic
conservative management
Conservative management includes:[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com
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]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com 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]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
Avoidance of metabolic disturbance (e.g., hypokalaemia, hyperkalaemia, metabolic acidosis)
Avoidance of alcohol intoxication.[18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
genetic counselling
Treatment recommended for ALL patients in selected patient group
Arrange genetic counselling for all patients to facilitate screening of relatives.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [80]Musunuru K, Hershberger RE, Day SM, et al. Genetic testing for inherited cardiovascular diseases: A scientific statement from the American Heart Association. Circ Genom Precis Med. 2020 Aug;13(4):e000067. https://www.ahajournals.org/doi/full/10.1161/HCG.0000000000000067?rfr_dat=cr_pub++0pubmed&url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org http://www.ncbi.nlm.nih.gov/pubmed/32698598?tool=bestpractice.com See Screening.
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com
Experience one or more episodes of documented spontaneous sustained ventricular tachycardia[7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com [68]Arnar DO, Mairesse GH, Boriani G, et al. Management of asymptomatic arrhythmias: a European Heart Rhythm Association (EHRA) consensus document, endorsed by the Heart Failure Association (HFA), Heart Rhythm Society (HRS), Asia Pacific Heart Rhythm Society (APHRS), Cardiac Arrhythmia Society of Southern Africa (CASSA), and Latin America Heart Rhythm Society (LAHRS). Europace. 2019 Mar 18;21(6):844–5. https://academic.oup.com/europace/article/21/6/844/5382236?login=false http://www.ncbi.nlm.nih.gov/pubmed/30882141?tool=bestpractice.com [91]National Institute for Health and Care Excellence. Implantable cardioverter defibrillators and cardiac resynchronisation therapy for arrhythmias and heart failure. Jun 2014 [internet publication]. https://www.nice.org.uk/guidance/ta314
Develop ventricular fibrillation during electrophysiological study with programmed ventricular stimulation.[7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
The patient’s life expectancy should also be taken into account; ICD may not be appropriate if meaningful survival is <1 year.[18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com
In general, ICDs should be avoided in patients without high risk features.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com The most significant of these is inhibition of the cardiac transient outward potassium current, which prolongs the refractory period.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com
Quinidine is useful to suppress ventricular arrhythmias.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com It should be considered in asymptomatic patients who:[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [5]Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7967791 http://www.ncbi.nlm.nih.gov/pubmed/31075787?tool=bestpractice.com
Have an ICD and experience recurrent shocks[7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com
Decline or are unsuitable for an ICD[7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
Have a spontaneous type I Brugada pattern on ECG[51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
Experience asymptomatic ventricular arrhythmia.[68]Arnar DO, Mairesse GH, Boriani G, et al. Management of asymptomatic arrhythmias: a European Heart Rhythm Association (EHRA) consensus document, endorsed by the Heart Failure Association (HFA), Heart Rhythm Society (HRS), Asia Pacific Heart Rhythm Society (APHRS), Cardiac Arrhythmia Society of Southern Africa (CASSA), and Latin America Heart Rhythm Society (LAHRS). Europace. 2019 Mar 18;21(6):844–5. https://academic.oup.com/europace/article/21/6/844/5382236?login=false http://www.ncbi.nlm.nih.gov/pubmed/30882141?tool=bestpractice.com
Monitor the patient's FBC for anaemia and thrombocytopenia.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com
Be aware that significant adverse effects and difficulty in obtaining the drug can limit its use.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [5]Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7967791 http://www.ncbi.nlm.nih.gov/pubmed/31075787?tool=bestpractice.com
Phosphodiesterase-III inhibitors (cilostazol or milrinone) may be considered for patients with BrS (as an alternative to quinidine), however, supportive data are limited.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com
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
conservative management
Conservative management includes:[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com
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]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com 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]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
Avoidance of metabolic disturbance (e.g., hypokalaemia, hyperkalaemia, metabolic acidosis)
Avoidance of alcohol intoxication.[18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
genetic counselling
Treatment recommended for ALL patients in selected patient group
Arrange genetic counselling for all patients to facilitate screening of relatives.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [80]Musunuru K, Hershberger RE, Day SM, et al. Genetic testing for inherited cardiovascular diseases: A scientific statement from the American Heart Association. Circ Genom Precis Med. 2020 Aug;13(4):e000067. https://www.ahajournals.org/doi/full/10.1161/HCG.0000000000000067?rfr_dat=cr_pub++0pubmed&url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org http://www.ncbi.nlm.nih.gov/pubmed/32698598?tool=bestpractice.com See Screening.
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com [91]National Institute for Health and Care Excellence. Implantable cardioverter defibrillators and cardiac resynchronisation therapy for arrhythmias and heart failure. Jun 2014 [internet publication]. https://www.nice.org.uk/guidance/ta314
The patient’s life expectancy should also be taken into account; ICD may not be appropriate if meaningful survival is <1 year.[18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com 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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com The most significant of these is inhibition of the cardiac transient outward potassium current, which prolongs the refractory period.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com
Quinidine is useful to suppress ventricular arrhythmias, and may also be used for medical management of atrial arrhythmias.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com It should be considered in patients who:[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [5]Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7967791 http://www.ncbi.nlm.nih.gov/pubmed/31075787?tool=bestpractice.com
Have an ICD and experience recurrent shocks[7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com
Decline, or are unsuitable for, an ICD[7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
Experience electrical storm (>2 episodes of ventricular tachycardia or ventricular fibrillation in 24 hours).[51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com See 'Acute ventricular arrhythmia' above.
Experience asymptomatic ventricular arrhythmia.[68]Arnar DO, Mairesse GH, Boriani G, et al. Management of asymptomatic arrhythmias: a European Heart Rhythm Association (EHRA) consensus document, endorsed by the Heart Failure Association (HFA), Heart Rhythm Society (HRS), Asia Pacific Heart Rhythm Society (APHRS), Cardiac Arrhythmia Society of Southern Africa (CASSA), and Latin America Heart Rhythm Society (LAHRS). Europace. 2019 Mar 18;21(6):844–5. https://academic.oup.com/europace/article/21/6/844/5382236?login=false http://www.ncbi.nlm.nih.gov/pubmed/30882141?tool=bestpractice.com
Monitor the patient's FBC for anaemia and thrombocytopenia.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com
Be aware that significant adverse effects and difficulty in obtaining the drug can limit its use.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [5]Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7967791 http://www.ncbi.nlm.nih.gov/pubmed/31075787?tool=bestpractice.com
Phosphodiesterase-III inhibitors (cilostazol or milrinone) may be considered for patients with BrS (as an alternative to quinidine), however, supportive data are limited.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com
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
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [5]Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7967791 http://www.ncbi.nlm.nih.gov/pubmed/31075787?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
Experience serious arrhythmic events despite optimised medical therapy[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [5]Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7967791 http://www.ncbi.nlm.nih.gov/pubmed/31075787?tool=bestpractice.com
Are intolerant to or would prefer not to have medical therapy, or medical therapy is ineffective[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [5]Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7967791 http://www.ncbi.nlm.nih.gov/pubmed/31075787?tool=bestpractice.com
Have spontaneous type 1 Brugada pattern on ECG and symptomatic ventricular arrhythmias who are unsuitable for, or decline an ICD[18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com
Have a history of electrical storms.[51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
Significant contraindications to ablation include:[38]Nademanee K, Veerakul G, Chandanamattha P, et al. Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium. Circulation. 2011;123(12):1270-9. https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.110.972612?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed http://www.ncbi.nlm.nih.gov/pubmed/21403098?tool=bestpractice.com
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com
conservative management
Conservative management includes:[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com
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]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com 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]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
Avoidance of metabolic disturbance (e.g., hypokalaemia, hyperkalaemia, metabolic acidosis)
Avoidance of alcohol intoxication.[18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
genetic counselling
Treatment recommended for ALL patients in selected patient group
Arrange genetic counselling for all patients to facilitate screening of relatives.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [80]Musunuru K, Hershberger RE, Day SM, et al. Genetic testing for inherited cardiovascular diseases: A scientific statement from the American Heart Association. Circ Genom Precis Med. 2020 Aug;13(4):e000067. https://www.ahajournals.org/doi/full/10.1161/HCG.0000000000000067?rfr_dat=cr_pub++0pubmed&url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org http://www.ncbi.nlm.nih.gov/pubmed/32698598?tool=bestpractice.com See Screening.
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com
Documented spontaneous sustained ventricular tachycardia with or without syncope.[7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
An ICD should be considered in patients who:
Have provoked type 1 Brugada pattern on ECG and a history of cardiogenic syncope[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com
Develop ventricular fibrillation during electrophysiological study with programmed ventricular stimulation.[7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
The patient’s life expectancy should also be taken into account; ICD may not be appropriate if meaningful survival is <1 year.[18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com 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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com The most significant of these is inhibition of the cardiac transient outward potassium current, which prolongs the refractory period.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com
Quinidine is useful to suppress ventricular arrhythmias, and may also be used for medical management of atrial arrhythmias.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com It should be considered in patients who:[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [5]Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7967791 http://www.ncbi.nlm.nih.gov/pubmed/31075787?tool=bestpractice.com
Have an ICD and experience recurrent shocks[7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com
Decline or are unsuitable for an ICD[7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
Experience electrical storm (>2 episodes of ventricular tachycardia or ventricular fibrillation in 24 hours)[51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com See 'Acute ventricular arrhythmia' above.
Experience asymptomatic ventricular arrhythmia.[68]Arnar DO, Mairesse GH, Boriani G, et al. Management of asymptomatic arrhythmias: a European Heart Rhythm Association (EHRA) consensus document, endorsed by the Heart Failure Association (HFA), Heart Rhythm Society (HRS), Asia Pacific Heart Rhythm Society (APHRS), Cardiac Arrhythmia Society of Southern Africa (CASSA), and Latin America Heart Rhythm Society (LAHRS). Europace. 2019 Mar 18;21(6):844–5. https://academic.oup.com/europace/article/21/6/844/5382236?login=false http://www.ncbi.nlm.nih.gov/pubmed/30882141?tool=bestpractice.com
Monitor the patient's FBC for anaemia and thrombocytopenia.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com
Be aware that significant adverse effects and difficulty in obtaining the drug can limit its use.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [5]Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7967791 http://www.ncbi.nlm.nih.gov/pubmed/31075787?tool=bestpractice.com
Phosphodiesterase-III inhibitors (cilostazol or milrinone) may be considered for patients with BrS (as an alternative to quinidine), however, supportive data are limited.[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com
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
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [5]Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7967791 http://www.ncbi.nlm.nih.gov/pubmed/31075787?tool=bestpractice.com [7]Zeppenfeld K, Tfelt-Hansen J, de Riva M, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Oct 21;43(40):3997-4126. https://academic.oup.com/eurheartj/article/43/40/3997/6675633?login=false http://www.ncbi.nlm.nih.gov/pubmed/36017572?tool=bestpractice.com [18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com [51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
Experience serious arrhythmic events despite optimized medical therapy[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [5]Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7967791 http://www.ncbi.nlm.nih.gov/pubmed/31075787?tool=bestpractice.com
Are intolerant to or would prefer not to have medical therapy, or medical therapy is ineffective[1]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com [5]Cronin EM, Bogun FM, Maury P, et al. 2019 HRS/EHRA/APHRS/LAHRS expert consensus statement on catheter ablation of ventricular arrhythmias. Europace. 2019 Aug 1;21(8):1143-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7967791 http://www.ncbi.nlm.nih.gov/pubmed/31075787?tool=bestpractice.com
Have spontaneous type 1 Brugada pattern on ECG and symptomatic ventricular arrhythmias who are unsuitable for or decline an ICD[18]Al-Khatib SM, Stevenson WG, Ackerman MJ, et al. 2017 AHA/ACC/HRS guideline for management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: Executive summary: A report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Heart Rhythm. 2018 Oct;15(10):e190-e252. https://www.heartrhythmjournal.com/article/S1547-5271(17)31249-3/fulltext http://www.ncbi.nlm.nih.gov/pubmed/29097320?tool=bestpractice.com
Have a history of electrical storms.[51]Priori SG, Wilde AA, Horie M, et al. Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm. 2013;10(12):e85-108. https://core.ac.uk/reader/82269654?utm_source=linkout http://www.ncbi.nlm.nih.gov/pubmed/23916535?tool=bestpractice.com
Significant contraindications to ablation include:[38]Nademanee K, Veerakul G, Chandanamattha P, et al. Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium. Circulation. 2011;123(12):1270-9. https://www.ahajournals.org/doi/10.1161/CIRCULATIONAHA.110.972612?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed http://www.ncbi.nlm.nih.gov/pubmed/21403098?tool=bestpractice.com
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]Krahn AD, Behr ER, Hamilton R, et al. Brugada syndrome. JACC Clin Electrophysiol. 2022 Mar;8(3):386-405. https://www.sciencedirect.com/science/article/pii/S2405500X2101080X?via%3Dihub http://www.ncbi.nlm.nih.gov/pubmed/35331438?tool=bestpractice.com
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