The true prevalence of Brugada syndrome (BrS) is difficult to determine because the diagnosis requires specific ECG findings (which are often intermittent), and many patients are asymptomatic.[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
[8]Benito B, Brugada J, Brugada R, Brugada P. Brugada syndrome. Rev Esp Cardiol. 2009;62(11):1297-315.
http://www.ncbi.nlm.nih.gov/pubmed/19889341?tool=bestpractice.com
[9]Antzelevitch C, Brugada P, Borggrefe M, et al. Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation. 2005;111(5):659-70.
https://www.ahajournals.org/doi/10.1161/01.CIR.0000152479.54298.51?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed
http://www.ncbi.nlm.nih.gov/pubmed/15655131?tool=bestpractice.com
However, prevalence of BrS with type 1 Brugada pattern on the ECG is thought to be around 1 in 2000.[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
[4]Bayés de Luna A, Brugada J, Baranchuk A, et al. Current electrocardiographic criteria for diagnosis of Brugada pattern: a consensus report. J Electrocardiol. 2012;45(5):433-42.
http://www.ncbi.nlm.nih.gov/pubmed/22920782?tool=bestpractice.com
[8]Benito B, Brugada J, Brugada R, Brugada P. Brugada syndrome. Rev Esp Cardiol. 2009;62(11):1297-315.
http://www.ncbi.nlm.nih.gov/pubmed/19889341?tool=bestpractice.com
[9]Antzelevitch C, Brugada P, Borggrefe M, et al. Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation. 2005;111(5):659-70.
https://www.ahajournals.org/doi/10.1161/01.CIR.0000152479.54298.51?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed
http://www.ncbi.nlm.nih.gov/pubmed/15655131?tool=bestpractice.com
[10]Jellins J, Milanovic M, Taitz DJ, Wan SH, Yam PW. Brugada syndrome. Hong Kong Med J. 2013;19(2):159-67.
https://www.hkmj.org/abstracts/v19n2/159.htm
http://www.ncbi.nlm.nih.gov/pubmed/23535677?tool=bestpractice.com
Prevalence of a type 2 or type 3 Brugada pattern on the ECG (which is not diagnostic of BrS, but requires further investigation) is around 1 in 500.[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
BrS is most commonly diagnosed in young- to middle-aged men; it is around 8 to 10 times more prevalent in men than in women.[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
[10]Jellins J, Milanovic M, Taitz DJ, Wan SH, Yam PW. Brugada syndrome. Hong Kong Med J. 2013;19(2):159-67.
https://www.hkmj.org/abstracts/v19n2/159.htm
http://www.ncbi.nlm.nih.gov/pubmed/23535677?tool=bestpractice.com
[11]Quan XQ, Li S, Liu R, Zheng K, Wu XF, Tang Q. A meta-analytic review of prevalence for Brugada ECG patterns and the risk for death. Medicine (Baltimore). 2016 Dec;95(50):e5643.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5268056
http://www.ncbi.nlm.nih.gov/pubmed/27977610?tool=bestpractice.com
The phenotypic expression of BrS appears to be age dependent because it is rare in children, with a prevalence of around 1 in 20,000.[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
[2]Oe H, Takagi M, Tanaka A, et al. Prevalence and clinical course of the juveniles with Brugada-type ECG in Japanese population. Pacing Clin Electrophysiol. 2005;28(6):549-54.
http://www.ncbi.nlm.nih.gov/pubmed/15955188?tool=bestpractice.com
[3]Yamakawa Y, Ishikawa T, Uchino K, et al. Prevalence of right bundle-branch block and right precordial ST-segment elevation (Brugada-type electrocardiogram) in Japanese children. Circ J. 2004;68(4):275-9.
http://www.ncbi.nlm.nih.gov/pubmed/15056820?tool=bestpractice.com
However, BrS is thought to be the cause of around 4% to 12% of sudden cardiac deaths in children and young athletes, and around 20% of sudden explained deaths in young people in general.[12]Behere SP, Weindling SN. Brugada syndrome in children - Stepping into unchartered territory. Ann Pediatr Cardiol. 2017 Sep-Dec;10(3):248-58.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5594936
http://www.ncbi.nlm.nih.gov/pubmed/28928611?tool=bestpractice.com
Note that children are not covered elsewhere in this topic.
BrS occurs more commonly in Asia than in Europe and the US.[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
[13]Mizusawa Y, Wilde AA. Brugada syndrome. Circ Arrhythm Electrophysiol. 2012;5(3):606-16.
https://www.ahajournals.org/doi/10.1161/CIRCEP.111.964577?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub%20%200pubmed
http://www.ncbi.nlm.nih.gov/pubmed/22715240?tool=bestpractice.com
It is particularly common in Southeast Asia, where nocturnal sudden death syndrome in young men was clinically reported by a variety of local names (e.g., bangungut, lai-tai, pokkuri, bei gui ya) in countries such as Philippines, Thailand, Japan, and China, prior to the formal classification of BrS.[14]Sarquella-Brugada G, Campuzano O, Arbelo E, Brugada J, Brugada R. Brugada syndrome: clinical and genetic findings. Genet Med. 2016;18(1):3-12.
https://www.gimjournal.org/article/S1098-3600(21)04299-4/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/25905440?tool=bestpractice.com
It is the leading cause of death in men under age 40 years in Southeast Asia.[4]Bayés de Luna A, Brugada J, Baranchuk A, et al. Current electrocardiographic criteria for diagnosis of Brugada pattern: a consensus report. J Electrocardiol. 2012;45(5):433-42.
http://www.ncbi.nlm.nih.gov/pubmed/22920782?tool=bestpractice.com