Brugada syndrome (BrS) is a genetic disorder in which the electrical activity of the heart is abnormal due to channelopathy.[2] It increases the risk of abnormal heart rhythms and sudden cardiac death.[2] Those affected may have episodes of syncope.[2] The abnormal heart rhythms seen in those with Brugada syndrome often occur at rest.[1][5] They may be triggered by a fever.[1][5]
About a quarter of those with Brugada syndrome have a family member who also has the condition.[2] Some cases may be due to a new genetic mutation or certain medications.[1] The most commonly involved gene is SCN5A which encodes the cardiac sodium channel.[6] Diagnosis is typically by electrocardiogram (ECG), however, the abnormalities may not be consistently present.[2] Medications such as ajmaline may be used to reveal the ECG changes.[2] Similar ECG patterns may be seen in certain electrolyte disturbances[7] or when the blood supply to the heart has been reduced.[8]
There is no cure for Brugada syndrome.[3] Those at higher risk of sudden cardiac death may be treated using an implantable cardioverter defibrillator (ICD).[4] In those without symptoms the risk of death is much lower, and how to treat this group is less clear.[3][9] Isoproterenol may be used in the short term for those who have frequent life-threatening abnormal heart rhythms, while quinidine may be used longer term.[3][10] Testing people's family members may be recommended.[3]
The condition affects between 1 and 30 per 10,000 people.[2] It is more common in males than females and in those of Asian descent.[1][2] The onset of symptoms is usually in adulthood.[2] It was firstly described by Andrea Nava and Bortolo Martini in Padova in 1989[11] but it is named after the Spanish cardiologists Pedro and Josep Brugada who described the condition in 1992.[3][12] Chen first described the genetic abnormality of SCN5A channels.[13]
^ abcdefg"Brugada syndrome". Genetics Home Reference. March 2015. Archived from the original on 28 October 2017. Retrieved 28 October 2017.
^ abcdefghijklmnoPolovina MM, Vukicevic M, Banko B, Lip GY, Potpara TS (October 2017). "Brugada syndrome: A general cardiologist's perspective". European Journal of Internal Medicine. 44: 19–27. doi:10.1016/j.ejim.2017.06.019. PMID 28645806.
^ abcdefgh"Brugada Syndrome". NORD (National Organization for Rare Disorders). 2016. Archived from the original on 11 February 2017. Retrieved 28 October 2017.
^ ab"Brugada syndrome". Genetic and Rare Diseases Information Center (GARD) – an NCATS Program. 2017. Archived from the original on 17 October 2017. Retrieved 28 October 2017.
^ abSarquella-Brugada G, Campuzano O, Arbelo E, Brugada J, Brugada R (January 2016). "Brugada syndrome: clinical and genetic findings". Genetics in Medicine. 18 (1): 3–12. doi:10.1038/gim.2015.35. PMID 25905440.
^Antzelevitch C, Patocskai B (January 2016). "Brugada Syndrome: Clinical, Genetic, Molecular, Cellular, and Ionic Aspects". Current Problems in Cardiology. 41 (1): 7–57. doi:10.1016/j.cpcardiol.2015.06.002. PMC 4737702. PMID 26671757.
^Doty B, Kim E, Phelps J, Akpunonu P (8 July 2020). "Pathophysiology of Hyperkalemia Presenting as Brugada Pattern on Electrocardiogram (ECG)". The American Journal of Case Reports. 21: e923464. doi:10.12659/AJCR.923464. ISSN 1941-5923. PMC 7370581. PMID 32636355.
^Antzelevitch C, Viskin S (2013). "Brugada Syndrome: Cellular Mechanisms and Approaches to Therapy". In Gussak I, Antzelevitch C, Wilde AA, Powell BD, Ackerman MJ, Shen WK (eds.). Electrical diseases of the heart. Basic foundations and primary electrical diseases. Vol. 1 (2nd ed.). London: Springer. pp. 497–536. ISBN 978-1-4471-4880-7. OCLC 841465583.
^Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, et al. (December 2013). "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. 10 (12): 1932–63. doi:10.1016/j.hrthm.2013.05.014. PMID 24011539.
^Belhassen B, Glick A, Viskin S (September 2004). "Efficacy of quinidine in high-risk patients with Brugada syndrome". Circulation. 110 (13): 1731–7. doi:10.1161/01.CIR.0000143159.30585.90. PMID 15381640.
^Cite error: The named reference Nava1989 was invoked but never defined (see the help page).
^Brugada P, Brugada J (November 1992). "Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report". Journal of the American College of Cardiology. 20 (6): 1391–6. doi:10.1016/0735-1097(92)90253-J. PMID 1309182.
^Cite error: The named reference Chen2018 was invoked but never defined (see the help page).
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