Brugada Syndrome

Syed Azhar Sherazi, Muhammad Rehan Khan

Abstract


The Brugada syndrome is a genetic disease that is characterized by abnormal electrocardiogram (ECG) findings and an increased risk of sudden cardiac death.1 it is named by the Spanish cardiologists Pedro Brugada and Joseph Brugada. It is the major cause of sudden unexplained death syndrome (SUDS), and is the most common cause of sudden death in young men without known underlying cardiac disease in Thailand and Laos.

Keywords


Electrocardiogram; Brugada Syndrome; Sudden cardiac death

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References


Frustaci A, Priori SG, Pieroni M, Chimenti C, Napolitano C, Rivolta I, et al. Cardiac histological substrate in patients with clinical phenotype of Brugada syndrome. Circulation. Dec 13 2005;112:3680-7.

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. Dec 1989;118:1203-9.

Brugada J, Brugada R, Brugada P. Determinants of sudden cardiac death in individuals with the electrocardiographic pattern of Brugada syndrome and no previous cardiac arrest. Circulation. Dec 23 2003;108:3092-6.

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. Nov 15 1992;20:1391-6.

Vorobiof G, Kroening D, Hall B, Brugada R, Huang D. Brugada syndrome with marked conduction disease: dual implications of a SCN5A mutation. Pacing Clin Electrophysiol. May 2008;31:630-4.

Kusano KF, Taniyama M, Nakamura K, Miura D, Banba K, Nagase S, et al. Atrial fibrillation in patients with Brugada syndrome relationships of gene mutation, electrophysiology, and clinical backgrounds. J Am Coll Cardiol 2008;51:1169-75.

Alings M, Wilde A. "Brugada" syndrome: clinical data and suggested pathophysiological mechanism. Circulation. Feb 9 1999;99:666-73.

Meregalli PG, Wilde AA, Tan HL. Pathophysiological mechanisms of Brugada syndrome: depolarization disorder, repolarization disorder, or more?. Cardiovasc Res. Aug 15 2005;67:367-78.

Nagase S, Kusano KF, Morita H, Nishii N, Banba K, Watanabe A, et al. Longer repolarization in the epicardium at the right ventricular outflow tract causes type 1 electrocardiogram in patients with Brugada syndrome. J Am Coll Cardiol. Mar 25 2008;51:1154-61.

Antzelevitch C, Brugada P, Brugada J, Brugada R. Brugada syndrome: from cell to bedside. Curr Probl Cardiol. Jan 2005;30:49-54.

Postema PG, van Dessel PF, Kors JA, Linnenbank AC, van Herpen G, Ritsema van Eck HJ, et al. Local depolarization abnormalities are the dominant pathophysiologic mechanism for type 1 electrocardiogram in brugada syndrome a study of electrocardiograms, vectorcardiograms, and body surface potential maps during ajmaline provocation. J Am Coll Cardiol 2010;55:789-97.

Kapplinger JD, Tester DJ, Alders M, Benito B, Berthet M, Brugada J, et al. An international compendium of mutations in the SCN5A-encoded cardiac sodium channel in patients referred for Brugada syndrome genetic testing. Heart Rhythm 2010;7:33-46.

Antzelevitch C, Pollevick GD, Cordeiro JM, Casis O, Sanguinetti MC, Aizawa Y, et al. Loss-of-function mutations in the cardiac calcium channel underlie a new clinical entity characterized by ST-segment elevation, short QT intervals, and sudden cardiac death. Circulation 2007;115:442-9.

London B, Michalec M, Mehdi H, Zhu X, Kerchner L, Sanyal S, et al. Mutation in glycerol-3-phosphate dehydrogenase 1 like gene (GPD1-L) decreases cardiac Na+ current and causes inherited arrhythmias. Circulation 2007;116:2260-8.

Watanabe H, Koopmann TT, Le Scouarnec S, Yang T, Ingram CR, Schott JJ, et al. Sodium channel beta1 subunit mutations associated with Brugada syndrome and cardiac conduction disease in humans. J Clin Invest 2008;118:2260-8.

Donohue D, Tehrani F, Jamehdor R, Lam C, Movahed MR. The prevalence of Brugada ECG in adult patients in a large university hospital in the western United States. Am Heart Hosp J 2008;6:48-50.

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;96:2595-600.

Bezzina CR, Shimizu W, Yang P, Koopmann TT, Tanck MW, Miyamoto Y, et al. Common sodium channel promoter haplotype in asian subjects underlies variability in cardiac conduction. Circulation 2006;113:338-44.

[Guideline] Antzelevitch C, Brugada P, Borggrefe M, Brugada J, Brugada R, Corrado D, et al. Brugada syndrome: report of the second consensus conference: endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation. Feb 8 2005;111:659-70.

Bordachar P, Reuter S, Garrigue S, Caï X, Hocini M, Jaïs P, et al. Incidence, clinical implications and prognosis of atrial arrhythmias in Brugada syndrome. Eur Heart J. May 2004;25:879-84.

Take Y, Morita H, Toh N, Nishii N, Nagase S, Nakamura K, et al. Identification of high-risk syncope related to ventricular fibrillation in patients with Brugada syndrome. Heart Rhythm 2012;9:752-9.

Wilde AA, Antzelevitch C, Borggrefe M, Brugada J, Brugada R, Brugada P, et al. Proposed diagnostic criteria for the Brugada syndrome: consensus report. Circulation 2002;106:2514-9.

Sangwatanaroj S, Prechawat S, Sunsaneewitayakul B, Sitthisook S, Tosukhowong P, Tungsanga K. New electrocardiographic leads and the procainamide test for the detection of the Brugada sign in sudden unexplained death syndrome survivors and their relatives. Eur Heart J 2001;22:2290-6.

Takagi M, Yokoyama Y, Aonuma K, Aihara N, Hiraoka M. Clinical characteristics and risk stratification in symptomatic and asymptomatic patients with brugada syndrome: multicenter study in Japan. J Cardiovasc Electrophysiol 2007;18:1244-51.

Junttila MJ, Brugada P, Hong K, Lizotte E, DE Zutter M, Sarkozy A, et al. Differences in 12-lead electrocardiogram between symptomatic and asymptomatic Brugada syndrome patients. J Cardiovasc Electrophysiol 2008;19:380-3.

Sarkozy A, Chierchia GB, Paparella G, Boussy T, De Asmundis C, Roos M, et al. Inferior and lateral electrocardiographic repolarization abnormalities in Brugada syndrome. Circ Arrhythm Electrophysiol 2009;2:154-61.

Kamakura S, Ohe T, Nakazawa K, Aizawa Y, Shimizu A, Horie M, et al. Long-term prognosis of probands with Brugada-pattern ST-elevation in leads V1-V3. Circ Arrhythm Electrophysiol 2009;2:495-503.

Zorzi A, Migliore F, Marras E, Marinelli A, Baritussio A, Allocca G, et al. Should all individuals with a nondiagnostic Brugada-electrocardiogram undergo sodium-channel blocker test?. Heart Rhythm 2012;9:909-16.

Brugada P, Geelen P, Brugada R, Mont L, Brugada J. Prognostic value of electrophysiologic investigations in Brugada syndrome. J Cardiovasc Electrophysiol 2001;12:1004-7.

Priori SG, Napolitano C, Gasparini M, Pappone C, Della Bella P, Giordano U, et al. Natural history of Brugada syndrome: insights for risk stratification and management. Circulation 2002;105:1342-7.

Gehi AK, Duong TD, Metz LD, Gomes JA, Mehta D. Risk stratification of individuals with the Brugada electrocardiogram: a meta-analysis. J Cardiovasc Electrophysiol 2006;17:577-83.

Probst V, Veltmann C, Eckardt L, Meregalli PG, Gaita F, Tan HL, et al. Long-term prognosis of patients diagnosed with Brugada syndrome: Results from the FINGER Brugada Syndrome Registry. Circulation 2010;121:635-43.

Priori SG, Gasparini M, Napolitano. Risk Stratification in Brugada Syndrome Results of the PRELUDE (PRogrammed ELectrical stimUlation preDictive valuE) Registry. J Am Coll Cardiol 2012;59:37-45.

Raju H, Papadakis M, Govindan M. Low prevalence of risk markers in cases of sudden death due to brugada syndrome relevance to risk stratification in brugada syndrome. J Am Coll Cardiol 2011;57:2340-5.

Antzelevitch C. The Brugada syndrome: ionic basis and arrhythmia mechanisms. J Cardiovascular Electrophysiology 2001; 12:268-72.

Márquez MF, Salica G, Hermosillo AG, Pastelín G, Gómez-Flores J, Nava S, et al. Ionic basis of pharmacological therapy in Brugada syndrome. J Cardiovasc Electrophysiol 2007;18:234-40.

Yang F, Hanon S, Lam P, Schweitzer P. Quinidine revisited. Am J Med 2009;122:317-21.

Postema PG, Wolpert C, Amin AS, Probst V, Borggrefe M, Roden DM, et al. Drugs and Brugada syndrome patients: review of the literature, recommendations, and an up-to-date. Heart Rhythm 2009;6:1335-41.


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© 2011 Gomal Journal of Medical Sciences