Brugada Syndrome: Diagnosis and Management
Abstract
Brugada syndrome associated with high incidence of sudden death in young and otherwise healthy adults in many parts of the world. The Brugada syndrome which is characterized by an ST-segment elevation in the right precordial ECG leads, is a familial disease that displays an autosomal dominant mode o? transmission with incomplete penetrance. The first and only gene to be linked to Brugada syndrome is SCN5A, the gene that encodes for the subunit of the cardiac sodium channel gene. More than 80 mutations in SCN5A have been linked to the syndrome.Based on ECG characteristics the syndrome divided into three types. Brugada syndrome is definitively diagnosed when a type 1 ST-segment elevation is observed in >1 right precordial lead (V1 to V3) in the presence or absence of a sodium channel– blocking agent, and in conjunction with one of the following: documented ventricular fibrillation (VF), polymorphic ventricular tachycardia (VT), a family history of sudden cardiac death at < 45 years old, coved-type ECGs in family members, inducibility of VT with programmed electrical stimulation, syncope, or nocturnal agonal respiration. Currently, an implantable cardioverter defibrilla?or (ICD) is the only proven effective treatment for the disease.
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References
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; 20:1391–1396.
Antzelevitch C, Brugada P, Brugada J, et al. Brugada syndrome: a decade of progress. Circ Res. 2002;91:1114 –1118.
Antzelevitch C, Arthur AM, Borggrefe M, et al. Proposed di-agnostic criteria for the brugada syndrome. Consensus Report. Circ.2002;106:2514-2519.
Antzelevitch C, Brugada P, Borggrefe M, et al. Brugada Syndrome : Report of the Second Consensus Conference. Circ.2005;111:659-670
Brugada R, Brugada J, Antzelevitch C, et al. Sodium Chan-nel Blockers Identify Risk for Sudden Death in Patients With ST-Segment Elevation and Right Bundle Branch Block but Structurally Normal Hearts. Circ.2000;101:510-515
Kannel WB, Cupples AL, D’Agostino RB. Sudden death risk in overt coronary heart disease: the Framingham study. Am Heart J. 1987;113: 799–804.
Anzelevith C. The Brugada syndrome: diagnostic criteria and cellular mechanism. Eur H Journal.2001;22:356-363
Hermida JS, Lemoine JL, Aoun FB, et al. Prevalence of the Brugada syndrome in an apparently healthy population. Am J Cardiol. 2000;86:91–94.
Sudden, unexpected, nocturnal deaths among Southeast Asian refugees. MMWR Morb Mortal Wkly Rep. 1981;30:581–584, 589.
Antzelevitch C. The Brugada syndrome: ionic basis and arrhythmia mechanisms. J Cardiovasc Electrophysiol. 2001;12:268–272.
Schulze-Bahr E, Eckardt L, Breithardt G, et al. Sodium channel gene (SCN5A) mutations in 44 index patients with Brugada syndrome: different incidences in familial and sporadic disease. Human Mut 2003;21:651–652.
Antzelevitch C, Brugada, P , Brugada J,et al. Brugada Syndrome: From Cell to Bedside. Curr Probl Cardiol. 2005 January ; 30(1): 9–54.
Herbert E, Chahine M. Clinical aspects and physiopathology of brugada syndrome:review of current concepts.Can. J. Physiol. Pharmacol.2006;84:795-802.
Litovsky SH, Antzelevitch C. Transient outward current promi-nent in canine ventricular epicardium but not endocardium. Circ Res. 1988;62: 116–126.
Di Diego JM, Sun ZQ, Antzelevitch C. Ito and action potential notch are smaller in left vs. right canine ventricular epicardium. Am J Physiol. 1996;271:H548–H561.
Di Diego JM, Antzelevitch C. High [Ca21]-induced electrical heterogeneity and extrasystolic activity in isolated canine ven-tricular epicardium: phase 2 reentry. Circulation. 1994;89:1839–1850.
Lukas A, Antzelevitch C. Phase 2 reentry as a mechanism of initiation of circus movement reentry in canine epicardium exposed to simulated ischemia: the antiarrhythmic effects of 4-aminopyridine. Cardiovasc Res. 1996;32:593– 603.
Yan GX, Antzelevitch C. Cellular basis for the Brugada Syndrome and other mechanisms of arrhythmogenesis associated with ST segment elevation. Circulation 1999; 100:1660–1666.
Lukas A, Antzelevitch C. Phase 2 reentry as a mechanism of initiation of circus movement reentry in canine epicardium exposed to simulated ischemia. The antiarrhythmic effects of 4-aminopyridine. Cardiovasc Res 1996; 32: 593–603.
Brugada P, Brugada J, Brugada R. Arrhythmia induction by antiarrhythmic drugs. Pacing Clin Electrophysiol. 2000;23:291–292.
Brugada R, Brugada J, Antzelevitch C, et al. Sodium channel blockers identify risk for sudden death in patients with ST-segment elevation and right bundle branch block but structurally normal hearts. Circulation. 2000;101:510 –515.
Shimizu W, Matsuo K, Takagi M, et al. Suyama K, Kurita T, Aihara N, Kamakura S. Body surface distribution and response to drugs of ST segment elevation in Brugada syndrome: clinical implication of eighty-seven-lead body surface potential map-ping and its application to twelve-lead electrocardiograms. J Cardiovasc Electrophysiol. 2000;11:396–404.
Sangwatanaroj S, Prechawat S, Sunsaneewitayakul B, et al. New electrocardiographic leads and the procainamide test for the detection of the Brugada sign in sudden unexplained death syn-drome survivors and their relatives. Eur Heart J. 2001;22:2290 –2296
Wolpert C, Constanze Echternach C, Veltmann C. Intrave-nous drug challenge using flecainide and ajmaline in patients with Brugada syndrome.Heart Rhythm. 2005 March ; 2(3): 254–260.
Shimizu W, Antzelevitch C, Suyama K, et al. Effect of sodium channel blockers on ST segment, QRS duration, and corrected QT interval in patients with Brugada syndrome. J Cardiovasc Electrophysiol. 2000;11:1320–1329.
Terajima K, Yamamoto T, Onodera H. Unmasking of Brugada Syndrome by an Antiarrhythmic Drug in a Patient with Septic Shock. Anesth Analg2006;102:233–236
Fish JM, Antzelevitch C, Role of Sodium and Calcium Channel Block in Unmasking the Brugada Syndrome. Heart Rhythm. 2004 July ; 1(2): 210–217.
Brugada J, Brugada R, Antzelevitch C, et al. Long-term follow-up of individuals with the electrocardiographic pattern of right bundle-branch block and ST-segment elevation in precordial leads V(1) to V(3). Circulation.2002;105:73–8.
Priori SG, Napolitano C, Gasparini M, et al. Natural history of Brugada syndrome: insights for risk stratification and manage-ment. Circulation.2002;105:1342–7.
Brugada J, Brugada R, Brugada P. Determinants of sudden car-diac death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest. Circulation. 2003;108:3092–6.
Brugada J, Brugada R, Brugada P. Pharmacological and device approach to therapy of inherited cardiac diseases associated with cardiac arrhythmias and sudden death. J Electrocardio.l2000;33 (Suppl):41–7.
Brugada P, Brugada R, Brugada J, Geelen P. Use of the prophy-lactic implantable cardioverter defibrillator for patients with normal hearts. Am J Cardiol.1999;83:98D–100D
Chalvidan T, Deharo JC, Dieuzaide P, et al.: Near fatal electrical storm in a patient equipped with an implantable cardioverter-defibrillator for Brugada syndrome. PACE. 2000;23:410-412
Ruskin JN: Idiopathic ventricular fibrillation: is there a role for electrophysiologic-guided antiarrhythmic drug therapy? J Cardiovasc Electrophysiol.1999;10:1313-1315
Belhassen B, Viskin S, Antzelevitch C: The Brugada syndrome: is implantable cardioverter defibrillator the only therapeutic option? PACE.2002;25:1634-1640
Brugada J, Brugada R, Brugada P. Right bundle-branch block and ST-segment elevation in leads V1 through V3: a marker for sudden death in patients without demonstrable structural heart disease. Circulation.1998;97:457– 460.
Antzelevitch C, Brugada P, Brugada J, et al. Clinical Approaches to Tachyarrhythmias. The Brugada Syndrome. Armonk, NY: Futura Publishing Co, 1999.
Alings M, Dekker L, Sadee A, Wilde A. Quinidine induced electrocardiographic normalization in two patients with Brugada syndrome. Pacing Clin Electrophysiol. 2001;24:1420 –1422
Tsuchiya T, Ashikaga K, Honda T, Arita M. Prevention of ventricular fibrillation by cilostazol, an oral phosphodiesterase inhibitor, in a patient with Brugada syndrome. J Cardiovasc Electrophysiol. 2002;13: 698–701
Antzelevitch C, Brugada P, Brugada J, et al. Brugada syndrome: a decade of progress. Circ Res. 2002;91:1114 –1118.
Antzelevitch C, Arthur AM, Borggrefe M, et al. Proposed di-agnostic criteria for the brugada syndrome. Consensus Report. Circ.2002;106:2514-2519.
Antzelevitch C, Brugada P, Borggrefe M, et al. Brugada Syndrome : Report of the Second Consensus Conference. Circ.2005;111:659-670
Brugada R, Brugada J, Antzelevitch C, et al. Sodium Chan-nel Blockers Identify Risk for Sudden Death in Patients With ST-Segment Elevation and Right Bundle Branch Block but Structurally Normal Hearts. Circ.2000;101:510-515
Kannel WB, Cupples AL, D’Agostino RB. Sudden death risk in overt coronary heart disease: the Framingham study. Am Heart J. 1987;113: 799–804.
Anzelevith C. The Brugada syndrome: diagnostic criteria and cellular mechanism. Eur H Journal.2001;22:356-363
Hermida JS, Lemoine JL, Aoun FB, et al. Prevalence of the Brugada syndrome in an apparently healthy population. Am J Cardiol. 2000;86:91–94.
Sudden, unexpected, nocturnal deaths among Southeast Asian refugees. MMWR Morb Mortal Wkly Rep. 1981;30:581–584, 589.
Antzelevitch C. The Brugada syndrome: ionic basis and arrhythmia mechanisms. J Cardiovasc Electrophysiol. 2001;12:268–272.
Schulze-Bahr E, Eckardt L, Breithardt G, et al. Sodium channel gene (SCN5A) mutations in 44 index patients with Brugada syndrome: different incidences in familial and sporadic disease. Human Mut 2003;21:651–652.
Antzelevitch C, Brugada, P , Brugada J,et al. Brugada Syndrome: From Cell to Bedside. Curr Probl Cardiol. 2005 January ; 30(1): 9–54.
Herbert E, Chahine M. Clinical aspects and physiopathology of brugada syndrome:review of current concepts.Can. J. Physiol. Pharmacol.2006;84:795-802.
Litovsky SH, Antzelevitch C. Transient outward current promi-nent in canine ventricular epicardium but not endocardium. Circ Res. 1988;62: 116–126.
Di Diego JM, Sun ZQ, Antzelevitch C. Ito and action potential notch are smaller in left vs. right canine ventricular epicardium. Am J Physiol. 1996;271:H548–H561.
Di Diego JM, Antzelevitch C. High [Ca21]-induced electrical heterogeneity and extrasystolic activity in isolated canine ven-tricular epicardium: phase 2 reentry. Circulation. 1994;89:1839–1850.
Lukas A, Antzelevitch C. Phase 2 reentry as a mechanism of initiation of circus movement reentry in canine epicardium exposed to simulated ischemia: the antiarrhythmic effects of 4-aminopyridine. Cardiovasc Res. 1996;32:593– 603.
Yan GX, Antzelevitch C. Cellular basis for the Brugada Syndrome and other mechanisms of arrhythmogenesis associated with ST segment elevation. Circulation 1999; 100:1660–1666.
Lukas A, Antzelevitch C. Phase 2 reentry as a mechanism of initiation of circus movement reentry in canine epicardium exposed to simulated ischemia. The antiarrhythmic effects of 4-aminopyridine. Cardiovasc Res 1996; 32: 593–603.
Brugada P, Brugada J, Brugada R. Arrhythmia induction by antiarrhythmic drugs. Pacing Clin Electrophysiol. 2000;23:291–292.
Brugada R, Brugada J, Antzelevitch C, et al. Sodium channel blockers identify risk for sudden death in patients with ST-segment elevation and right bundle branch block but structurally normal hearts. Circulation. 2000;101:510 –515.
Shimizu W, Matsuo K, Takagi M, et al. Suyama K, Kurita T, Aihara N, Kamakura S. Body surface distribution and response to drugs of ST segment elevation in Brugada syndrome: clinical implication of eighty-seven-lead body surface potential map-ping and its application to twelve-lead electrocardiograms. J Cardiovasc Electrophysiol. 2000;11:396–404.
Sangwatanaroj S, Prechawat S, Sunsaneewitayakul B, et al. New electrocardiographic leads and the procainamide test for the detection of the Brugada sign in sudden unexplained death syn-drome survivors and their relatives. Eur Heart J. 2001;22:2290 –2296
Wolpert C, Constanze Echternach C, Veltmann C. Intrave-nous drug challenge using flecainide and ajmaline in patients with Brugada syndrome.Heart Rhythm. 2005 March ; 2(3): 254–260.
Shimizu W, Antzelevitch C, Suyama K, et al. Effect of sodium channel blockers on ST segment, QRS duration, and corrected QT interval in patients with Brugada syndrome. J Cardiovasc Electrophysiol. 2000;11:1320–1329.
Terajima K, Yamamoto T, Onodera H. Unmasking of Brugada Syndrome by an Antiarrhythmic Drug in a Patient with Septic Shock. Anesth Analg2006;102:233–236
Fish JM, Antzelevitch C, Role of Sodium and Calcium Channel Block in Unmasking the Brugada Syndrome. Heart Rhythm. 2004 July ; 1(2): 210–217.
Brugada J, Brugada R, Antzelevitch C, et al. Long-term follow-up of individuals with the electrocardiographic pattern of right bundle-branch block and ST-segment elevation in precordial leads V(1) to V(3). Circulation.2002;105:73–8.
Priori SG, Napolitano C, Gasparini M, et al. Natural history of Brugada syndrome: insights for risk stratification and manage-ment. Circulation.2002;105:1342–7.
Brugada J, Brugada R, Brugada P. Determinants of sudden car-diac death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest. Circulation. 2003;108:3092–6.
Brugada J, Brugada R, Brugada P. Pharmacological and device approach to therapy of inherited cardiac diseases associated with cardiac arrhythmias and sudden death. J Electrocardio.l2000;33 (Suppl):41–7.
Brugada P, Brugada R, Brugada J, Geelen P. Use of the prophy-lactic implantable cardioverter defibrillator for patients with normal hearts. Am J Cardiol.1999;83:98D–100D
Chalvidan T, Deharo JC, Dieuzaide P, et al.: Near fatal electrical storm in a patient equipped with an implantable cardioverter-defibrillator for Brugada syndrome. PACE. 2000;23:410-412
Ruskin JN: Idiopathic ventricular fibrillation: is there a role for electrophysiologic-guided antiarrhythmic drug therapy? J Cardiovasc Electrophysiol.1999;10:1313-1315
Belhassen B, Viskin S, Antzelevitch C: The Brugada syndrome: is implantable cardioverter defibrillator the only therapeutic option? PACE.2002;25:1634-1640
Brugada J, Brugada R, Brugada P. Right bundle-branch block and ST-segment elevation in leads V1 through V3: a marker for sudden death in patients without demonstrable structural heart disease. Circulation.1998;97:457– 460.
Antzelevitch C, Brugada P, Brugada J, et al. Clinical Approaches to Tachyarrhythmias. The Brugada Syndrome. Armonk, NY: Futura Publishing Co, 1999.
Alings M, Dekker L, Sadee A, Wilde A. Quinidine induced electrocardiographic normalization in two patients with Brugada syndrome. Pacing Clin Electrophysiol. 2001;24:1420 –1422
Tsuchiya T, Ashikaga K, Honda T, Arita M. Prevention of ventricular fibrillation by cilostazol, an oral phosphodiesterase inhibitor, in a patient with Brugada syndrome. J Cardiovasc Electrophysiol. 2002;13: 698–701
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How to Cite
Octavianus, R., & Yuniadi, Y. (1). Brugada Syndrome: Diagnosis and Management. Indonesian Journal of Cardiology, 33(2), 113-26. https://doi.org/10.30701/ijc.v33i2.62
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