The numerous denominations of the Brugada syndrome and proposal about how to put an end to an old controversy - a historical-critical perspective


  • Joseane Elza Tonussi Mendes Laboratorio de Delineamento de Estudos e Escrita Científica, Centro Universitário Saúde ABC, Santo André, São Paulo, Brazil
  • Kjell Nikus Heart Center, Tampere University Hospital and Faculty of Medicine and Health Technology, Tampere University, Finland
  • Raimundo Barbosa-Barros Coronary Center of the Hospital de Messejana Dr. Carlos Alberto Studart Gomes, Fortaleza, Ceará, Brazil.
  • Andrés Ricardo Pérez-Riera Laboratorio de Delineamento de Estudos e Escrita Científica, Centro Universitário Saúde ABC, Santo André, São Paulo, Brazil



Brugada syndrome, eponymous, nomenclature, electrocardiographic hallmark


Backgroung: The eponymous Brugada Syndrome (BrS) in honor of its discovery as an independent entity by the Spanish/ Catalan Brugada brothers, Pedro and Josep, has deserved numerous denominations derived mainly from the clinical genotype/phenotype correlation. The purpose of this manuscript is to present and analyze the nomenclatures that this intriguing and challenging syndrome has received over the past 28 years. We also compared the main features between cases from the first report of the Brugada brothers and an article by Martini et al. The nomenclatures used by these authors are closely linked to the BrS, but the cases (except one) presented in the article by Martini et al do not present the type 1 Brugada ECG pattern, which is mandatory for the diagnosis of BrS.


Download data is not yet available.


Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: a distinct clinical and electrocardiographic syndrome. A multicenter report. J Am Coll Cardiol. 1992;20(6):1391-6.

Yan GX, Antzelevitch C. Cellular basis for the electrocardiographic J wave. Circulation. 1996;93(2):372-9.

Kobayashi T, Shintani U, Yamamoto T, Shida S, Isshiki N, Tanaka T, et al. Familial occurrence of electrocardiographic abnormalities of the Brugada-type. Intern Med. 1996;35(8):637-40.

Chen Q, Kirsch GE, Zhang D, Brugada R, Brugada J, Brugada P, et al. Genetic basis and molecular mechanism for idiopathic ventricular fibrillation. Nature. 1998;392(6673):293-6.

Chokevivat V, Warintrawat S, Choprapawan C. Epidemiology of Lai Tai in Thailand. Desire Press. 1993:38–50.

Baron RC, Thacker SB, Gorelkin L, Vernon AA, Taylor WR, Choi K. Sudden death among Southeast Asian refugees. An unexplained nocturnal phenomenon. JAMA. 1983;250(21):2947-51.

Leads from the MMWR. Update: sudden unexplained death syndrome among Southeast Asian refugees--United States. JAMA. 1988;260(14):2033.

Centers for Disease C. Sudden, unexpected, nocturnal deaths among Southeast Asian refugees. MMWR Morb Mortal Wkly Rep. 1981;30(47):581-4, 9.

Parrish RG, Tucker M, Ing R, Encarnacion C, Eberhardt M. Sudden unexplained death syndrome in Southeast Asian refugees: a review of CDC surveillance. MMWR CDC Surveill Summ. 1987;36(1):43SS53SS.

Goh KT, Chao TC, Chew CH. Sudden nocturnal deaths among Thai construction workers in Singapore. Lancet. 1990;335(8698):1154.

Nademanee K, Veerakul G, Nimmannit S, Chaowakul V, Bhuripanyo K, Likittanasombat K, et al. Arrhythmogenic marker for the sudden unexplained death syndrome in Thai men. Circulation. 1997;96(8):2595-600.

Nademanee K, Raju H, de Noronha SV, Papadakis M, Robinson L, Rothery S, et al. Fibrosis, Connexin-43, and Conduction Abnormalities in the Brugada Syndrome. J Am Coll Cardiol. 2015;66(18):1976-86.

Gaw AC, Lee B, Gervacio-Domingo G, Antzelevitch C, Divinagracia R, Jocano F, Jr. Unraveling the Enigma of Bangungut: Is Sudden Unexplained Nocturnal Death Syndrome (SUNDS) in the Philippines a Disease Allelic to the Brugada Syndrome? Philipp J Intern Med. 2011;49(3):165-76.

Vatta M, Dumaine R, Varghese G, Richard TA, Shimizu W, Aihara N, et al. Genetic and biophysical basis of sudden unexplained nocturnal death syndrome (SUNDS), a disease allelic to Brugada syndrome. Hum Mol Genet. 2002;11(3):337-45.

Guazon MP. Algunas notas sobre bangungut [in Spanish]. Revista Filipina de Medicina Y Farmacia. 1917;8:437–42.

Okada R, Kawai S. Histopathology of the conduction system in sudden cardiac death. Jpn Circ J. 1983;47(5):573-80.

Takeichi S, Nakajima K, Nakajima Y, Fujita MQ. Pathological characteristics of Pokkuri Death Syndrome; narrow circumferences of the coronary arteries in Pokkuri Death Syndrome cases. Atherosclerosis. 2008;200(1):80-2.

Marcus FI, Fontaine GH, Guiraudon G, Frank R, Laurenceau JL, Malergue C, et al. Right ventricular dysplasia: a report of 24 adult cases. Circulation. 1982;65(2):384-98.

Corrado D, Nava A, Buja G, Martini B, Fasoli G, Oselladore L, et al. Familial cardiomyopathy underlies syndrome of right bundle branch block, ST segment elevation and sudden death. J Am Coll Cardiol. 1996;27(2):443-8.

Ohe T. Idiopathic ventricular fibrillation of the Brugada type: an atypical form of arrhythmogenic right ventricular cardiomyopathy? Intern Med. 1996;35(8):595.

Coronel R, Casini S, Koopmann TT, Wilms-Schopman FJ, Verkerk AO, de Groot JR, et al. Right ventricular fibrosis and conduction delay in a patient with clinical signs of Brugada syndrome: a combined electrophysiological, genetic, histopathologic, and computational study. Circulation. 2005;112(18):2769-77.

Frustaci A, Russo MA, Chimenti C. Structural myocardial abnormalities in asymptomatic family members with Brugada syndrome and SCN5A gene mutation. Eur Heart J. 2009;30(14):1763.

Dhamapurkar SK, Wilson BA, Rose A, Florschutz G. Brugada syndrome and the story of Dave. Neuropsychol Rehabil. 2018;28(2):259-67.

Huang L, Tang S, Chen Y, Zhang L, Yin K, Wu Y, et al. Molecular pathological study on LRRC10 in sudden unexplained nocturnal death syndrome in the Chinese Han population. Int J Legal Med. 2017;131(3):621-8.

Ikeda T, Abe A, Yusu S, Nakamura K, Ishiguro H, Mera H, et al. The full stomach test as a novel diagnostic technique for identifying patients at risk of Brugada syndrome. J Cardiovasc Electrophysiol. 2006;17(6):602-7.

Zhao Q, Chen Y, Peng L, Gao R, Liu N, Jiang P, et al. Identification of rare variants of DSP gene in sudden unexplained nocturnal death syndrome in the southern Chinese Han population. Int J Legal Med. 2016;130(2):317-22.

Zheng J, Zheng D, Su T, Cheng J. Sudden Unexplained Nocturnal Death Syndrome: The Hundred Years’ Enigma. J Am Heart Assoc. 2018;7(5).

Shimizu A, Zankov DP, Sato A, Komeno M, Toyoda F, Yamazaki S, et al. Identification of transmembrane protein 168 mutation in familial Brugada syndrome. FASEB J. 2020.

Le Scouarnec S, Karakachoff M, Gourraud JB, Lindenbaum P, Bonnaud S, Portero V, et al. Testing the burden of rare variation in arrhythmia-susceptibility genes provides new insights into molecular diagnosis for Brugada syndrome. Hum Mol Genet. 2015;24(10):2757-63.

Saito YA, Strege PR, Tester DJ, Locke GR, 3rd, Talley NJ, Bernard CE, et al. Sodium channel mutation in irritable bowel syndrome: evidence for an ion channelopathy. Am J Physiol Gastrointest Liver Physiol. 2009;296(2):G211-8.

Perez-Riera AR, Baranchuk A, Zhang L, Barbosa-Barros R, de Abreu LC, Brugada P. Myotonic dystrophy and Brugada syndrome: A common pathophysiologic pathway? J Electrocardiol. 2017;50(4):513-7.

Noebels JL. Sodium channel gene expression and epilepsy. Novartis Found Symp. 2002;241:109-20;discussion 20-3, 226-32.

Priest BT, Garcia ML, Middleton RE, Brochu RM, Clark S, Dai G, et al. A disubstituted succinamide is a potent sodium channel blocker with efficacy in a rat pain model. Biochemistry. 2004;43(30):9866-76.

Vanmolkot KR, Babini E, de Vries B, Stam AH, Freilinger T, Terwindt GM, et al. The novel p.L1649Q mutation in the SCN1A epilepsy gene is associated with familial hemiplegic migraine: genetic and functional studies. Mutation in brief #957. Online. Hum Mutat. 2007;28(5):522.

Aiba T. Recent understanding of clinical sequencing and gene-based risk stratification in inherited primary arrhythmia syndrome. J Cardiol. 2019;73(5):335-42.

Campuzano O, Sarquella-Brugada G, Fernandez-Falgueras A, Cesar S, Coll M, Mates J, et al. Genetic interpretation and clinical translation of minor genes related to Brugada syndrome. Hum Mutat. 2019;40(6):749-64.

Gussak I, Bjerregaar PB. History of the Brugada syndrome. In: Antzelevitch C, Brugada P, Brugada J, Brugada R, editors. The Brugada Syndrome From Bench to Bedside. USA: Blackwell Futura; 2005

Martini B, Nava A, Thiene G, Buja GF, Canciani B, Scognamiglio R, et al. Ventricular fibrillation without apparent heart disease: description of six cases. Am Heart J. 1989;118(6):1203-9.

Wilde AAM, Amin AS. Clinical Spectrum of SCN5A Mutations: Long QT Syndrome, Brugada Syndrome, and Cardiomyopathy. JACC Clin Electrophysiol. 2018;4(5):569-79.

Corrado D, Basso C, Buja G, Nava A, Rossi L, Thiene G. Right bundle branch block, right precordial stsegment elevation, and sudden death in young people. Circulation. 2001;103(5):710-7.

Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, 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;10(12):1932-63.

Havakuk O, Viskin S. A Tale of 2 Diseases: The History of Long-QT Syndrome and Brugada Syndrome. J Am Coll Cardiol. 2016;67(1):100-8.

Corrado D, Basso C, Thiene G. Sudden cardiac death in young people with apparently normal heart. Cardiovasc Res. 2001;50(2):399-408.

Kataoka N, Nagase S, Kamakura T, Nakajima K, Wada M, Yamagata K, et al. Clinical Differences in Japanese Patients Between Brugada Syndrome and Arrhythmogenic Right Ventricular Cardiomyopathy With Long-Term Follow-Up. Am J Cardiol. 2019;124(5):715-22.

Calo L, Giustetto C, Martino A, Sciarra L, Cerrato N, Marziali M, et al. A New Electrocardiographic Marker of Sudden Death in Brugada Syndrome: The S-Wave in Lead I. J Am Coll Cardiol. 2016;67(12):1427-40.

Perez-Riera AR, Ferreira Filho C, de Abreu LC, Ferreira C, Yanowitz FG, Femenia F, et al. Do patients with electrocardiographic Brugada type 1 pattern have associated right bundle branch block? A comparative vectorcardiographic study. Europace. 2012;14(6):889-97.