Atrial arrhythmias occur in about 10% of patients with Brugada syndrome.[61]Giustetto C, Cerrato N, Gribaudo E, et al. Atrial fibrillation in a large population with Brugada electrocardiographic pattern: prevalence, management, and correlation with prognosis. Heart Rhythm. 2014;11(2):259-65.
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[93]Sacher F, Probst V, Iesaka Y, et al. Outcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: a multicenter study. Circulation. 2006;114(22):2317-24.
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[94]Kewcharoen J, Rattanawong P, Kanitsoraphan C, et al. Atrial fibrillation and risk of major arrhythmic events in Brugada syndrome: A meta-analysis. Ann Noninvasive Electrocardiol. 2019 Nov;24(6):e12676.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6931417
http://www.ncbi.nlm.nih.gov/pubmed/31353765?tool=bestpractice.com
Brugada syndrome (BrS) is the cause of around 28% of cases of sudden cardiac death in patients with an apparently normal heart and around 5% to 10% of cases of resuscitated cardiac arrest.[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
[91]Papadakis M, Papatheodorou E, Mellor G, et al. The diagnostic yield of Brugada syndrome after sudden death with normal autopsy. J Am Coll Cardiol. 2018 Mar 20;71(11):1204-14.
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[92]van der Werf C, Hofman N, Tan HL, et al. Diagnostic yield in sudden unexplained death and aborted cardiac arrest in the young: the experience of a tertiary referral center in The Netherlands. Heart Rhythm. 2010;7(10):1383-1389
http://www.ncbi.nlm.nih.gov/pubmed/20646679?tool=bestpractice.com
Around 1 in 25 patients with BrS have a history of cardiac arrest at time of initial diagnosis.[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
[54]Casado-Arroyo R, Berne P, Rao JY, Rodriguez-Mañero M, et al. Long-term trends in newly diagnosed Brugada syndrome: Implications for risk stratification. J Am Coll Cardiol. 2016 Aug 9;68(6):614-23.
https://pubmed.ncbi.nlm.nih.gov/27491905
http://www.ncbi.nlm.nih.gov/pubmed/27491905?tool=bestpractice.com
Incidence of sudden cardiac death in those without an ICD is approximately 0.19%.[83]Probst V, Goronflot T, Anys S, et al. Robustness and relevance of predictive score in sudden cardiac death for patients with Brugada syndrome. Eur Heart J. 2021 May 1;42(17):1687-95.
https://academic.oup.com/eurheartj/article/42/17/1687/6027696?login=false
http://www.ncbi.nlm.nih.gov/pubmed/33289793?tool=bestpractice.com
Patients who have BrS and cardiogenic syncope have an annual incidence of a serious arrhythmic event of about 1.4%.[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 likely mechanism of serious arrhythmic events is ventricular fibrillation initiated by short-coupled premature ventricular complexes.[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
Patients who require an ICD tend to be <50 years old and are at risk of ICD-related complications (e.g., lead fracture, infection) and unnecessary ICD shocks. One meta-analysis found a 4.5% annual rate of ICD-related complications and a 3.5% annual rate of inappropriate shocks.[95]Dereci A, Yap SC, Schinkel AFL. Meta-analysis of clinical outcome after implantable cardioverter-defibrillator implantation in patients with Brugada syndrome. JACC Clin Electrophysiol. 2019 Feb;5(2):141-8.
https://www.sciencedirect.com/science/article/pii/S2405500X18307424?via%3Dihub
http://www.ncbi.nlm.nih.gov/pubmed/30784682?tool=bestpractice.com