Guideline sources The following summarized guidelines for the evaluation and management of brugada syndrome (BS) are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC 2022; 2018), the American Heart Association (AHA/HRS/ACC 2018), the Asia Pacific Heart Rhythm Society (APHRS/EHRA/HRS 2013), and the European Heart Rhythm Association (EHRA/HRS 2011). 1 2 3 4 5 Calculator NYHA functional classification f Guidelines 1. Screening and diagnosis Diagnosis: https://web.pathway.md/diseases/recoFegDbEM4Q3Udl 1/5 6/29/23, 3:21 AM Brugada syndrome Pathway As per ESC 2022 guidelines, diagnose BS in patients with spontaneous type 1 Brugada ECG pattern and no other heart disease. B Show 3 more As per EHRA 2013 guidelines: Diagnose BS in patients with ST-segment elevation with type 1 morphology 2 mm in 1 lead among the right precordial leads V1, V2, positioned in the 2nd, 3rd, or 4th intercostal space occurring either spontaneously or after provocative drug test with IV administration of class I antiarrhythmic drugs. B Diagnose BS in patients with type 2 or type 3 ST-segment elevation in 1 lead among the right precordial leads V1, V2 positioned in the 2nd, 3rd, or 4th intercostal space when a provocative drug test with IV administration of class I antiarrhythmic drugs induces a type I ECG morphology. B 2. Diagnostic investigations Drug challenge testing: As per ESC 2022 guidelines, do not obtain sodium channel blocker testing in patients with a prior type 1 Brugada pattern. D As per AHA 2018 guidelines, consider obtaining pharmacological challenge testing with a sodium channel blocker in patients with suspected BS in the absence of spontaneous type 1 Brugada ECG pattern. C Genetic testing: As per ESC 2022 guidelines, obtain genetic testing for SCN5A gene mutation in probands with BS. B As per AHA 2018 guidelines: Consider providing genetic counseling and obtaining genetic testing to facilitate cascade screening of relatives in patients with suspected or established BS. C Provide genetic counseling and obtaining mutation-specific genetic testing in first-degree relatives of patients with a causative mutation for BS. B As per HRS 2011 guidelines, obtain mutation-specific genetic testing in family members and appropriate relatives following the identification of BS-causative mutation in an index case. A Show 2 more 3. Diagnostic procedures Implantable loop recorder: consider implanting a loop recorder in patients with BS with unexplained syncope. C Electrophysiology study: https://web.pathway.md/diseases/recoFegDbEM4Q3Udl 2/5 6/29/23, 3:21 AM Brugada syndrome Pathway As per ESC 2022 guidelines, consider performing programmed electrical stimulation in asymptomatic patients with a spontaneous type I BS ECG pattern. C As per AHA 2018 guidelines, consider obtaining an electrophysiological study with programmed ventricular stimulation using single and double extra stimuli for further risk stratification in patients with asymptomatic BS and spontaneous type 1 Brugada ECG pattern. C As per ESC 2018 guidelines, consider obtaining an invasive electrophysiology study in patients with Brugada ECG pattern and syncope of suspected arrhythmic etiology. C 4. Medical management Avoidance of aggravating factors: As per ESC 2022 guidelines, avoid using drugs likely to induce ST-segment elevation in right precordial leads in patients with BS. D Show 2 more As per EHRA 2013 guidelines, avoid using drugs likely to induce or aggravate ST-segment elevation in right precordial leads in patients with BS. D Show 2 more Watchful waiting: offer observation without therapy in asymptomatic patients with only inducible type 1 Brugada ECG pattern. B Quinidine: As per ESC 2022 guidelines, consider initiating quinidine in patients with BS qualifying for an ICD but having a contraindication, declining, or having recurrent ICD shocks. C As per AHA 2018 guidelines: Offer intensification of therapy with quinidine or catheter ablation in patients with BS experiencing recurrent ICD shocks for polymorphic VT. B Initiate quinidine or offer catheter ablation in patients with spontaneous type 1 Brugada ECG pattern and symptomatic ventricular arrhythmia either not eligible for or declining ICD placement. B As per ESC 2013 guidelines, consider initiating quinidine in patients with BS and a history of arrhythmic storms defined as > 2 episodes of VT/VF in 24 hours. C Show 2 more Isoproterenol infusion: As per ESC 2022 guidelines, consider administering isoproterenol infusion in patients with BS in electrical storm. C As per EHRA 2013 guidelines, consider administering isoproterenol infusion for the management of arrhythmic storms in patients with BS. C 5. Therapeutic procedures https://web.pathway.md/diseases/recoFegDbEM4Q3Udl 3/5 6/29/23, 3:21 AM Brugada syndrome Pathway Implantable cardioverter-defibrillator: As per ESC 2022 guidelines, perform ICD placement in patients with BS with a history of any of the following: aborted cardiac arrest documented spontaneous sustained VT. B Show 2 more As per AHA 2018 guidelines: Perform ICD in patients with a cardiac channelopathy and sudden cardiac arrest, if the expected meaningful survival is > 1 year. B Perform ICD in patients with BS with spontaneous type 1 Brugada ECG pattern survived cardiac arrest, sustained ventricular arrhythmia, or with a recent history of syncope presumed due to ventricular arrhythmia, if the expected meaningful survival is > 1 year. B As per ESC 2018 guidelines: Consider performing ICD placement in patients with Brugada ECG pattern and syncope of suspected arrhythmic etiology. C Do not perform ICD placement in patients with Brugada ECG pattern and reflex-mediated syncope in the absence of other risk factors. D As per EHRA 2013 guidelines, perform ICD placement in patients with BS with any of the following: cardiac arrest documented spontaneous sustained VT with or without syncope. A Show 3 more Catheter ablation: As per ESC 2022 guidelines: Consider performing catheter ablation of triggering premature ventricular contractions and/or RV outflow tract epicardial substrate in patients with BS with recurrent appropriate ICD shocks refractory to drug therapy. C Do not perform catheter ablation in asymptomatic patients with BS. D As per AHA 2018 guidelines: Perform catheter ablation or offer intensification of therapy with quinidine in patients with BS experiencing recurrent ICD shocks for polymorphic VT. B Perform catheter ablation or initiate quinidine in patients with spontaneous type 1 Brugada ECG pattern and symptomatic ventricular arrhythmia either not eligible for or declining ICD placement. B As per EHRA 2013 guidelines, consider performing catheter ablation in patients with BS and a history of arrhythmic storms or repeated appropriate ICD shocks. C Clinical findings https://web.pathway.md/diseases/recoFegDbEM4Q3Udl 4/5 6/29/23, 3:21 AM Brugada syndrome Pathway Symptoms Cardiac exam Chest discomfort Cardiac arrest Dyspnea Genetic testing Palpitations Seizure Scn5a gene mutation Syncope ECG findings St-segment elevation VF VT References 1. Sana M Al-Khatib, William G Stevenson, Michael J Ackerman et al. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines and the Heart Rhythm Society. Circulation. 2018 Sep 25;138 13):e272-e391. Open 2. Silvia G Priori, Arthur A Wilde, Minoru Horie et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm. 2013 Dec;10 12 1932 63. Open 3. Katja Zeppenfeld, Jacob Tfelt-Hansen, Marta de Riva et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J. 2022 Aug 26;ehac262. Open 4. Michele Brignole, Angel Moya, Frederik J de Lange et al. 2018 ESC Guidelines for the diagnosis and management of syncope. Eur Heart J. 2018 Jun 1;39 21 1883 1948. Open 5. Michael J Ackerman, Silvia G Priori, Stephan Willems et al. HRS/EHRA expert consensus statement on the state of genetic testing for the channelopathies and cardiomyopathies this document was developed as a partnership between the Heart Rhythm Society HRS and the European Heart Rhythm Association EHRA . Heart Rhythm. 2011 Aug;8 8 1308 39. Open https://web.pathway.md/diseases/recoFegDbEM4Q3Udl 5/5