Brugada syndrome is still a current clinical problem. What do we know today - a literature review
DOI:
https://doi.org/10.12775/JEHS.2023.46.01.012Keywords
sudden cardiac death, Brugada syndrome, ventricular arrhythmiaAbstract
Brugada syndrome (BrS) is a rare congenital arrhythmia syndrome. BrS is characterized by a tendency to syncope, ventricular arrhythmias and sudden cardiac death. These symptoms occur mainly at night and can occur spontaneously or as a result of provocation by, for example, fever, stress or medication. Based on the differences in electrocardiography, three types were distinguished. Genetic studies are being conducted, which have shown a significant defect of the SCN5A gene, which in turn leads to disturbances in the functioning of the sodium channel NaV1.5 has a negative impact on the course of depolarization of myocardial cells. Patients with symptoms of the disease are subjected to implantation of a cardioverter-defibrillator, which protects them against sudden death. Data was obtained from the PubMed and Google Scholar platforms.
References
Adler A. Brugada syndrome: diagnosis, risk stratifica- tion, and management. Curr Opin Cardiol. 2016; 31(1):37–45.
Brugada J, Campuzano O, Arbelo E, et al. Present status of brugada syndrome: JACC state-of-the–art review. J Am Coll Cardiol. 2018;72(9):1046–1059.
Polovina MM, Vukicevic M, Banko B, et al. Brugada syndrome: a general cardiologist’s perspective. Eur J Intern Med. 2017;44:19–27.
Brugada R, Campuzano O, Sarquella-Brugada G, et al. Brugada syndrome. Methodist Debakey Cardiovasc J. 2014;10(1):25–28.
Vohra J, Rajagopalan S. Update on the diagnosis and management of brugada syndrome. Heart Lung Circ. 2015;24(12):1141–1148.
Corrado D, Migliore F, Zorzi A. Brugada syndrome: in search of a cause. J Am Coll Cardiol. 2018;72(22): 2758–2760.
Sieira J, Dendramis G, Brugada P. Pathogenesis and management of Brugada syndrome. Nat Rev Cardiol. 2016;13(12):744–756.
Elizari MV, Levi R, Acunzo RS, et al. Abnormal expres- sion of cardiac neural crest cells in heart develop- ment: a different hypothesis for the etiopathogenesis of brugada syndrome. Heart Rhythm. 2007;4(3):359–365.
Sroubek J, Probst V, Mazzanti A, et al. Programmed ventricular stimulation for risk stratification in the brugada syndrome: a pooled analysis. Circulation. 2016;133(7):622–630.
Wu W, Tian L, Ke J, et al. Risk factors for cardiac events in patients with Brugada syndrome: a PRISMA-compliant meta-analysis and systematic review. Medicine (Baltimore). 2016;95(30):e4214.
Conte G, de Asmundis C, Sieira J, et al. Prevalence and clinical impact of early repolarization pattern and qrs-fragmentation in high-risk patients with bru- gada syndrome. Circ J. 2016;80(10):2109–2116.
Postępowanie u chorych z komorowymi zaburzeniami rytmu serca i zapobieganie nagłemu zgonowi sercowemu - Podsumowanie wytycznych European Society of Cardiology 2022
https://medtube.pl/kardiologia/zdjecia-medyczne/592-ekg-zespol-brugadow
Kołodziej M, Sledź J, Janion M. Zespół Brugadów--niedoceniana przyczyna zgonów u osób bez organicznej choroby serca [Brugada syndrome--underestimated cause of sudden cardiac death in patients without an organic cardiac disease--a case report]. Kardiol Pol. 2009 Feb;67(2):159-61. Polish. PMID: 19288378.
Skawski W, Bąkowski D, Wożakowska-Kapłon B. Zespół Brugadów-długa droga do prawidłowego rozpoznania. Choroby Serca i Naczyń 2013 Tom 10, Nr 6, 332-336
Takenaka S, Kusano KF, Hisamtsu K, et al. Relatively benign clinical course in asymptomatic patient with Brugada-type electrocardiogram without family history of sudden death. J Cardiovasc Electrophysiol 2001; 12: 2-8.
Pérez-Hernández M, Matamoros M, Alfayate S, Nieto-Marín P, Utrilla RG, Tinaquero D, de Andrés R, Crespo T, Ponce-Balbuena D, Willis BC, Jiménez-Vazquez EN, Guerrero-Serna G, da Rocha AM, Campbell K, Herron TJ, Díez-Guerra FJ, Tamargo J, Jalife J, Caballero R, Delpón E. Brugada syndrome trafficking-defective Nav1.5 channels can trap cardiac Kir2.1/2.2 channels. JCI Insight. 2018 Sep 20;3(18):e96291. doi: 10.1172/jci.insight.96291. PMID: 30232268; PMCID: PMC6237221.
Chakrabarti S, Wu X, Yang Z, Wu L, Yong SL, Zhang C, Hu K, Wang QK, Chen Q. MOG1 rescues defective trafficking of Na(v)1.5 mutations in Brugada syndrome and sick sinus syndrome. Circ Arrhythm Electrophysiol. 2013 Apr;6(2):392-401. doi: 10.1161/CIRCEP.111.000206. Epub 2013 Feb 18. PMID: 23420830; PMCID: PMC3633223.
Tadros R, Tan HL; ESCAPE-NET Investigators; El Mathari S, Kors JA, Postema PG, Lahrouchi N, Beekman L, Radivojkov-Blagojevic M, Amin AS, Meitinger T, Tanck MW, Wilde AA, Bezzina CR. Predicting cardiac electrical response to sodium-channel blockade and Brugada syndrome using polygenic risk scores. Eur Heart J. 2019 Oct 1;40(37):3097-3107. doi: 10.1093/eurheartj/ehz435. PMID: 31504448; PMCID: PMC6769824.
Wijeyeratne YD, Tanck MW, Mizusawa Y, Batchvarov V, Barc J, Crotti L, Bos JM, Tester DJ, Muir A, Veltmann C, Ohno S, Page SP, Galvin J, Tadros R, Muggenthaler M, Raju H, Denjoy I, Schott JJ, Gourraud JB, Skoric-Milosavljevic D, Nannenberg EA, Redon R, Papadakis M, Kyndt F, Dagradi F, Castelletti S, Torchio M, Meitinger T, Lichtner P, Ishikawa T, Wilde AAM, Takahashi K, Sharma S, Roden DM, Borggrefe MM, McKeown PP, Shimizu W, Horie M, Makita N, Aiba T, Ackerman MJ, Schwartz PJ, Probst V, Bezzina CR, Behr ER. SCN5A Mutation Type and a Genetic Risk Score Associate Variably With Brugada Syndrome Phenotype in SCN5A Families. Circ Genom Precis Med. 2020 Dec;13(6):e002911. doi: 10.1161/CIRCGEN.120.002911. Epub 2020 Nov 9. PMID: 33164571; PMCID: PMC7748043.
Bezzina CR, Barc J, Mizusawa Y, Remme CA, Gourraud JB, Simonet F, Verkerk AO, Schwartz PJ, Crotti L, Dagradi F, Guicheney P, Fressart V, Leenhardt A, Antzelevitch C, Bartkowiak S, Borggrefe M, Schimpf R, Schulze-Bahr E, Zumhagen S, Behr ER, Bastiaenen R, Tfelt-Hansen J, Olesen MS, Kääb S, Beckmann BM, Weeke P, Watanabe H, Endo N, Minamino T, Horie M, Ohno S, Hasegawa K, Makita N, Nogami A, Shimizu W, Aiba T, Froguel P, Balkau B, Lantieri O, Torchio M, Wiese C, Weber D, Wolswinkel R, Coronel R, Boukens BJ, Bézieau S, Charpentier E, Chatel S, Despres A, Gros F, Kyndt F, Lecointe S, Lindenbaum P, Portero V, Violleau J, Gessler M, Tan HL, Roden DM, Christoffels VM, Le Marec H, Wilde AA, Probst V, Schott JJ, Dina C, Redon R. Common variants at SCN5A-SCN10A and HEY2 are associated with Brugada syndrome, a rare disease with high risk of sudden cardiac death. Nat Genet. 2013 Sep;45(9):1044-9. doi: 10.1038/ng.2712. Epub 2013 Jul 21. Erratum in: Nat Genet. 2013 Nov;45(11):1409. Borggrefe, Martin [added]; Schimpf, Rainer [added]. PMID: 23872634; PMCID: PMC3869788.
Milman A, Andorin A, Gourraud JB, Sacher F, Mabo P, Kim SH, Maeda S, Takahashi Y, Kamakura T, Aiba T, Conte G, Juang JJM, Leshem E, Rahkovich M, Hochstadt A, Mizusawa Y, Postema PG, Arbelo E, Huang Z, Denjoy I, Giustetto C, Wijeyeratne YD, Napolitano C, Michowitz Y, Brugada R, Casado-Arroyo R, Champagne J, Calo L, Sarquella-Brugada G, Tfelt-Hansen J, Priori SG, Takagi M, Veltmann C, Delise P, Corrado D, Behr ER, Gaita F, Yan GX, Brugada J, Leenhardt A, Wilde AAM, Brugada P, Kusano KF, Hirao K, Nam GB, Probst V, Belhassen B. Age of First Arrhythmic Event in Brugada Syndrome: Data From the SABRUS (Survey on Arrhythmic Events in Brugada Syndrome) in 678 Patients. Circ Arrhythm Electrophysiol. 2017 Dec;10(12):e005222. doi: 10.1161/CIRCEP.117.005222. Epub 2017 Dec 18. PMID: 29254945.
Y. Michowitz, A. Milman, A. Andorin, et al. Characterization and management of arrhythmic events in young patients with Brugada syndrome. J Am Coll Cardiol, 73 (2019), pp. 1756-1765
M.C. Gonzalez Corcia, J. Sieira, A. Sarkozy, et al. Brugada syndrome in the young: an assessment of risk factors predicting future events Europace, 19 (2017), pp. 1864-1873
L.R. Olde Nordkamp, A.S. Vink, A.A. Wilde, et al. Syncope in Brugada syndrome: prevalence, clinical significance, and clues from history taking to distinguish arrhythmic from nonarrhythmic causes. Heart Rhythm, 12 (2015), pp. 367-375
C. Antzelevitch, G.X. Yan, M.J. Ackerman, et al. J-Wave syndromes expert consensus conference report: emerging concepts and gaps in knowledge. Heart Rhythm, 13 (2016), pp. e295-e324
T. Miyazaki, H. Mitamura, S. Miyoshi, K. Soejima, Y. Aizawa, S. Ogawa. Autonomic and antiarrhythmic drug modulation of ST segment elevation in patients with Brugada syndrome. J Am Coll Cardiol, 27 (1996), pp. 1061-1070
C.C. Cheung, G. Mellor, M.W. Deyell, et al. Comparison of ajmaline and procainamide provocation tests in the diagnosis of Brugada syndrome. J Am Clin Cardiol EP, 5 (2019), pp. 504-512
A.D. Krahn, M. Gollob, R. Yee, et al. Diagnosis of unexplained cardiac arrest: role of adrenaline and procainamide infusion. Circulation, 112 (2005), pp. 2228-2234
M. Lei, L. Wu, D.A. Terrar, C.L. Huang. Modernized classification of cardiac antiarrhythmic drugs. Circulation, 138 (2018), pp. 1879-1896
E.M. Vaughan Williams. A classification of antiarrhythmic actions reassessed after a decade of new drugs. J Clin Pharmacol, 24 (1984), pp. 129-147
L. Vitali Serdoz, H. Rittger, F. Furlanello, D. Bastian. Quinidine-A legacy within the modern era of antiarrhythmic therapy. Pharmacological Res, 144 (2019), pp. 257-263
B. Belhassen, A. Glick, S. Viskin. Efficacy of quinidine in high-risk patients with Brugada syndrome. Circulation, 110 (2004), pp. 1731-1737
J.S. Hermida, I. Denjoy, J. Clerc, et al. Hydroquinidine therapy in Brugada syndrome. J Am Coll Cardiol, 43 (2004), pp. 1853-1860
M. Haïssaguerre, F. Extramiana, M. Hocini, et al. Mapping and ablation of ventricular fibrillation associated with long-QT and Brugada syndromes. Circulation, 108 (2003), pp. 925-928
J. Brugada, C. Pappone, A. Berruezo, et al. Brugada syndrome phenotype elimination by epicardial substrate ablation. Circ Arrhythmia Electrophysiol, 8 (2015), pp. 1373-1381
G.C. Fernandes, A. Fernandes, R. Cardoso, et al. Ablation strategies for the management of symptomatic Brugada syndrome: a systematic review. Heart Rhythm, 15 (2018), pp. 1140-1147
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