--- abstract: | The Brugada disease, the last clinico-cardiologic entity described in the 20th century, initially called right bundle branch block syndrome with ST segment elevation from V1 to V2 or V3 and sudden cardiac death, is genetically determined in a dominant autosomal mode, and it affects the alpha subunit of the Na+ channel by alteration of chromosome 3 and mutation in the SCN5A gene. In clinical diagnosis the mentioned electrocardiographic pattern in a patient without structural heart disease and positivity in pharmacological tests are considered major criteria. As minor criteria, the following are considered: positive family history, presence of syncope with unknown origin, documented episode of VT/VF, inducibility in electrophysiologic study and positivity of genetic study. The long-standing technology of ECG, with more than a century of existence, remains as the supplementary method with highest value in diagnosis, and currently new electrocardiographic criteria are suggested, which indicate high risk of VF. Natural history indicates a somber diagnosis in symptomatic patients with a high index of arrhythmic SCD secondary to very fast polymorphic ventricular tachycardia bursts, which degenerate into VF. Asymptomatic individuals with only a Brugada-type electrocardiographic pattern have a low risk. The prognosis seems to depend more on clinical facts, since a positive electrophysiologic study has an accuracy of just around 50%. We propose that this entity should be promoted to the category of disease, since it has a characteristic set of signs and symptoms, and an identified genetic defect. altloc: - http://www.ipej.org/0304/riera.htm chapter: ~ commentary: ~ commref: ~ confdates: ~ conference: ~ confloc: ~ contact_email: ~ creators_id: [] creators_name: - family: Riera given: Andrés Ricardo Pérez honourific: '' lineage: '' - family: Schapachnik given: Edgardo honourific: '' lineage: '' - family: Ferreira given: Celso honourific: '' lineage: '' date: 2003-10 date_type: published datestamp: 2005-04-20 department: ~ dir: disk0/00/00/42/51 edit_lock_since: ~ edit_lock_until: ~ edit_lock_user: ~ editors_id: [] editors_name: - family: Singh given: Balbir honourific: '' lineage: '' - family: Lokhandwala given: Yash honourific: '' lineage: '' - family: Francis given: Johnson honourific: '' lineage: '' - family: Gupta given: Anup honourific: '' lineage: '' eprint_status: archive eprintid: 4251 fileinfo: /style/images/fileicons/text_html.png;/4251/1/riera.htm full_text_status: public importid: ~ institution: ~ isbn: ~ ispublished: pub issn: ~ item_issues_comment: [] item_issues_count: 0 item_issues_description: [] item_issues_id: [] item_issues_reported_by: [] item_issues_resolved_by: [] item_issues_status: [] item_issues_timestamp: [] item_issues_type: [] keywords: 'Brugada Disease, Brugada Syndrome, "prehistory," history' lastmod: 2011-03-11 08:55:58 latitude: ~ longitude: ~ metadata_visibility: show note: ~ number: 4 pagerange: 253-260 pubdom: TRUE publication: Indian Pacing and Electrophysiology Journal publisher: Indian Pacing and Electrophysiology Group refereed: TRUE referencetext: |- 1. Osher HL, Wolff L. Electrocardiographic patern simulating acute myocardial injury. J Med Sci 1953; 226:541-545. 2. Calo AA, The triad secondary R wave, RS-T segment elevation and T waves inversion in rigth precordial leads: a normal electrocardiographic variant. G Ital Cardiol 1975;5:955-960. 3. Vatta M,Dumaine R Varghese G, Richard TA, Shimizu W, Aihara N, Nademanee K, Brugada R, Brugada J, Veerakul G, Li H, Bowles NE, Brugada P, Antzelevitch C, Towbin JA. Genetic and biophysical basis of sudden unexplained nocturnal death syndrome (SUNDS), a disease allelic to Brugada syndrome. Hum Mol Genet 2002 1;11:337-345. 4. Martini B, Nava A, Thiene G, et al. Ventricular fibrillation without apparent heart disease: description of six cases. Am Heart J 1989; 116:1203-1209. 5. Brugada P, Brugada J. A distinct clinical and electrocardiographic syndrome: right bundle branch block, persistent ST segment elevation with normal QT interval and sudden cardiac death (abstr). PACE 1991;14:746. 6. Brugada P, Brugada J. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: A distinct clinical and electrocardiographic syndrome. J Am Coll Cardiol 1992, 20: 1391-1396. 7. Pérez Riera AR, Fortunato de Cano S, Fleury de Padua Neto LA, Schapachnik E. Síndrome de Brugada: Nuevos conceptos y expectativas futuras. Rev Argent Cardiol 2001;69:652-662. 8. Sumiyoshi M, Nakata Y, Hisaoka T. A case of idiopathic ventricular fibrillation with incomplete right bundle branch block and persistent ST segment elevation Jpn Heart J 1993, 34: 661-666 9. Proclemer A, Facchin D, Feruglio GA, Nucifora R. Fibrillazione ventricolare recidivante, blocco di branca destra, persistent sopraslivellamento del tratto ST in V1-V3: una nuova syndroma aritmica? Descrizione di un caso clinico. G Ital Cardiol 1993, 23: 1211-1218. 10. Nacarella F. Malignant ventricular arrhythmias in patients with a right bundle branch block and persistent ST segment elevation in V1-V3: A probable arrhythmogenic cardiomyopathy of the right ventricle. G Ital Cardiol 1993, 23: 1219-1222. 11. Martini B, Nava A, Canciani B, Thiene G. Right bundle branch block, persistent ST segment elevation and sudden cardiac death. J Am Coll Cardiol 1993; 22:633 (letter). 12. Brugada P, Brugada J. Do not get confused, please. G Ital Cardiol 1993 (letter). 13. Ferracci An, Fromer M, Schlaepfer J, Pruvot E, Kappenberger l. Fibrillation ventriculaire primitive et rècidive précoce. A propos d’un cas associant bloc de branche droit et sus-décalage persistant du segment ST. Arch Mal Coeur 1994, 87: 1359-1362. 14. Bjerregaard P, Gussak I, Kotar SL, et al. Recurrent syncope in a patient with prominent J wave. Am Heart J 1994, 127:1426-1430. 15. Tada H, Aihara N, Ohe T, et al. New insights in idiopathic ventricular fibrillation patients with right precordial ST segment elevation. Circulation (abstract) 1995, 4: 281. 16. Tohyou Y, Nakazawa K, Ozawa A, et al. A survey in the incidence of rigth bundle branch block with ST segment elevarion among normal population. Jpn J Electrocardiol 1995;15:223-226. 17. D’Onofrio A, Cuomo S, Musto B, Boccalatte A. Right bundle branch block, persistent ST segment elevation in V1-V3 and sudden cardiac death: always a distinct syndrome? G Ital Cardiol 1995;25:1171-1175. 18. Yan G-X, Antzelevitch C. Cellular basis for the electrocardiographic J wave. Circulation 1996, 93: 372-379. 19. Kobayashi T, Shintani U, Yamamoto T, et al. Familial occurrence of electrocardiographic abnormalities of the Brugada-type. Intern Med 1996;35:637-640. 20. Miyazaki T, Mitamura H, Miyoshi S, Soejima K, Aizawa Y, Ogawa S. Autonomic and antiarrhythmic modulation of ST segment elevation in patients with Brugada syndrome. J Am Coll Cardiol 1996, 27: 1061-1070. 21. Shimada M, Miyazaki T, Miyoshi S, et al. Sustained monomorphic ventricular tachycardia in a patient with Brugada syndrome. Jpn Circ J 1996;60:364-370. 22. Martini B, Nava A, Thiene G, Buja GF, Canciani B, Scognamiglio R, Daliento L, Dalla Volta S. Ventricular fibrillation without apparent heart disease: description of six cases. Am Heart J 1989;116:1203-1209. 23. Antzelevitch C, Brugada P, Brugada J, Brugada R, Shimizu W, Gussak I, Pérez Riera AR. Brugada Sindrome A Decade of Progress. Circ Res. 2002;91:1114-1118. 24. Antzelevitch C, Brugada P, Brugada J, Brugada R, Towbin JA, Nademanee K. Brugada syndrome: 1992-2002: a historical perspective. J Am Coll Cardiol. 2003;41:1665-1671. 25. Corrado D, Nava A, Buja G, et al. Familial cardiomyopathy underlies syndrome of right bundle branch block, ST segment elevation and sudden death. J Am Coll Cardiol 1996, 27: 443-448. 26. Ohe T. Idiopathic ventricular fibrillation of the Brugada type: an atypical form of arrhythmogenic right ventricular cardiomyopathy? Intern Med 1996;35:595 (editorial.) 27. Fontaine G. Familial cardiomyopathy associated with right bundle branch block, ST segment elevation and sudden death. J Am Coll Cardiol 1996;28:540-541 (letter). 28. Kobayashi H, Ohonish S, Momosi M, Kusakabe K, Okawa T. Characteristic findings of I-123MIBG scintigraphy in patients with Brugada syndrome Circulation (abstract) 1997; p 152. 29. Agostini D, Scanu P, Loiselet P, et al. Iodine-123metaiodobenzylguanindine SPECT of regional cardiac adrenergic denervation in Brugada syndrome. J Nucl Med 1998; 39:1129-1132. 30. Riemann R, Matheja P, Wichter T, et al. Locally reduced cardiac I-123-meta-iodobenzylguanidine (I-12MIBG)uptake in Brugada syndrome. 5th International Conference of Nuclear Cardiology (ICNC5) Viena Austria MAY 2 - 5, 2001 HOFDURG CENTER. 31. Wichter T, Matheja P, Eckardt L, et al. Cardiac autonomic dysfunction in Brugada syndrome. Circulation 2002;105:702-706. 32. Chinushi M, Aizawa Y, Ogawa Y, Shiba M, Takahashi K. Discrepant drug action of disopyramide on ECG abnormalities and induction of ventricular arrhythmias in a patient with Brugada syndrome Journal of Electrocardiography 1997; 30: 133-134. 33. Chen Q, Kirsch GE, Zhang D, Brugada R, Brugada P, Brugada J, et al. Genetic basis and molecular mechanisms for idiopathic ventricular fibrillation. Nature 1998;392:293-296. 34. Nakamura W, Segawa K, Ito H, Tanaka S, Yoshimmoto N. Class Ic antiarrhythmic drugs, flecainide and pilsicainide, produce ST segment elevation simulating inferior myocardial ischemia. J Cardiovasc Electrophysiol 1998;9:855-858. 35. Makita N, Shirai N. Mutant cardiac Na+ channel alpha subunit (T1620M) of an idiopathic ventricular fibrillation family exhibits altered functional association with beta1 subunit. Circulation 1998, abstract 275. 36. Tagaki M, Aihara N, Taguchi A, et al. Localized right ventricular morphological abnormalities in patients with Brugada syndrome by ultrafast computed tomography and magnetic resonance imaging. Is Brugada syndrome truly idiopathic? Circulation 1998, abstract 848. 37. Balser JR. Sodium "channelopathies" and sudden death: Must you be so sensitive? Circ Res 1999;85:872-874. 38. Bezzina C, Veldkamp MW, van den Berg MP, et al. A single Na+ channel mutation causing both long-QT and Brugada syndrome. Circ Res 1999;85:1206-1213. 39. Ikeda T, Sakata T, Sakabe K, et al. Noninvasive risk stratification markers in the Brugada syndrome: Comparison of late potentials, T-wave alternans and QT dispersion. PACE 2000;23:731. 40. Ikeda T, Sakurada H. and Sakabe K. et al. Assessment of noninvasive markers in identifying patients at risk in the Brugada Syndrome: insight into risk stratification. J Am Coll Cardiol 2001, 37:1628-1634. 41. Priori SG, Gasparini M, Napolitano C, et al. Clinical and genetic heterogeneity of the right bundle branch block and ST segment elevation syndrome. A prospective evaluation of 52 families. Circulation 2000;102:2509-2515. 42. Nishizaki M, Sakurada H, Ashikaga T, et al. Effect of insulin on ST segment elevation in Brugada syndrome. Circulation 2000;102:585. 43. Gussak I, Hammill SC.: Clinical diagnosis and risk stratification in patients with Brugada syndrome J Am Coll Cardiol 2001;37:1635-1638. 44. Vatta M,Dumaine R Varghese G, Richard TA, Shimizu W, Aihara N, Nademanee K, Brugada R, Brugada J, Veerakul G, Li H, Bowles NE, Brugada P, Antzelevitch C, Towbin JA. Genetic and biophysical basis of sudden unexplained nocturnal death syndrome (SUNDS), a disease allelic to Brugada syndrome. Hum Mol Genet 2002 1;11:337-345. 45. Pérez Riera AR, Schapachnik E, VIRTUAL SYMPOSIUM ABOUT THE BRUGADA SYNDROME: TEN YEARS OF HISTORY: 1992/2002 http://www.brugada-symposium.org/ 46. Atarashi H, Ogawa S, For The Idiopathic Ventricular Fibrillation Investigators. New ECG Criteria for High-Risk Brugada Syndrome. Circ J 2003 ;67:8-10. relation_type: [] relation_uri: [] reportno: ~ rev_number: 8 series: ~ source: ~ status_changed: 2007-09-12 16:57:35 subjects: - ipej succeeds: ~ suggestions: ~ sword_depositor: ~ sword_slug: ~ thesistype: ~ title: |- Brugada Disease: Chronology Of Discovery And Paternity. Preliminary Observations And Historical Aspects type: journalp userid: 4424 volume: 3