medigraphic.com
ISSN 1561-3011 (Electronic)

2012, Number 2

<< Back Next >>

Rev Cubana Invest Bioméd 2012; 31 (2)

Update on the management of patients with the Brugada syndrome ECG pattern

Castro HJA

Language: Spanish
References: 46
Page: 175-186
PDF size: 465.03 Kb.


ABSTRACT

The Brugada syndrome is a channelopathy causing between 4-12 % of all sudden cardiac deaths and 20 % in subjects with a "healthy heart". It is a clinical and electrocardiographic syndrome characterized by a convex electrocardiographic pattern in at least 2 right precordial leads as well a propensity to the occurrence of syncopes and/or cardiac arrest caused by polymorphic ventricular tachycardia and/or ventricular fibrillation. Malignant ventricular arrhythmias are caused by the transmural dispersion of repolarization and/or delayed conduction in the right ventricular outflow tract. It is more common in the male sex, frequently appearing in mid life. The risk of new events in symptomatic subjects is 30-40% in the 3 years following diagnosis. The treatment of choice is implantation of an automatic defibrillator. Quinidine has been used in patients with implanted defibrillators and frequent events of ventricular arrhythmia, as well as in those refusing to have such devices implanted. Management of asymptomatic subjects is under discussion. The database at the cardiac arrhythmia service of the Institute of Cardiology contains data for more than 80 patients with this syndrome, 45 with implanted automatic defibrillators.


REFERENCES

  1. Podrid PJ, Myerburg RJ. Epidemiology and stratification of risk for sudden cardiac death. Clin Cardiol. 2005;28:I3-11.

  2. Lloyd-Jones D, Adams RJ, Brown TM, Carnethon M, Dai S, De Simone G, et al. Heart disease and stroke statistics-2010 update: a report from the American Heart Association. Circulation. 2010;121:e46-215.

  3. Zipes DP, Wellens HJ. Sudden cardiac death. Circulation. 1998;98:2334-51.

  4. Antzelevitch C, Brugada P, Brugada J, Brugada R, Shimizu W, Gussak I, et al. Brugada syndrome: a decade of progress. Circ Res. 2002;91:1114-8.

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

  6. García-Castro M, García C, Reguero JR, Miara A, Rubín JM, Álvarez V, y cols. Espectro mutacional del gen SCN5A en pacientes españoles con síndrome de Brugada. Rev Esp Cardiol. 2010;63:856-9.

  7. 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;11:337-45.

  8. Priori SG, Napolitano C, Gasparini M, Pappone C, Della BP, Brignole M, et al. Clinical and genetic heterogeneity of right bundle branch block and ST-segment elevation syndrome: a prospective evaluation of 52 families. Circulation. 2000;102:2509-15.

  9. Brugada J, Brugada R, Antzelevitch C, Towbin J, Nademanee K, Brugada P. Long-term follow-up of individuals with the electrocardiographic pattern of right bundle-branch block and ST-segment elevation in precordial leads V1 to V3. Circulation. 2002;105:73-8.

  10. 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;392:293-6.

  11. Tester DJ, Ackerman MJ. Cardiomyopathies/channelopathies in clinical practice genetic testing for potentially lethal, highly treatable inherited. Circulation. 2011;123:1021-37.

  12. Monteforte N, Napolitano C, Priori S. Genética y arritmias: aplicaciones diagnósticas y pronósticas. Rev Esp Cardiol. 2012;65:27886.

  13. Yan GX, Antzelevitch C. Cellular basis for the Brugada syndrome and other mechanisms of arrhythmogenesis associated with ST segment elevation. Circulation. 1999;100:1660-6.

  14. Pitzalis MV, Anaclerio M, Iacoviello M, Forleo C, Guida P, Troccoli R, et al. QT-interval prolongation in right precordial leads: an additional electrocardiographic hallmark of Brugada syndrome. J Am Coll Cardiol. 2003;42:1632-7.

  15. Kanda M, Shimizu W, Matsuo K, Nagaya N, Taguchi A, Suyama K, et al. Electrophysiologic characteristics and implications of induced ventricular fibrillation in symptomatic patients with Brugada syndrome. J Am Coll Cardiol. 2002;39:1799-805.

  16. Postema PG, Van Dessel PM, 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.

  17. Nademanee K, Veerakul G, Chandanamattha P, Chaothawee L, Ariyachaipanich A, Jirasirirojanakorn K, et al. Prevention of ventricular fibrillation episodes in Brugada syndrome by catheter ablation over the anterior right ventricular outflow tract epicardium. Circulation. 2011;123;1270-9.

  18. Wilde AM, 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.

  19. Brugada P. Commentary on the Brugada ECG pattern: A marker of channelopathy, structural heart disease, or neither?. Toward a unifying mechanism of the Brugada syndrome. Circ Arrhythm Electrophysiol. 2010;3:280-2.

  20. Hirata K, Takagi Y, Nakada M, Kyushima M, Asato H. Beat-to-beat variation of the ST segment in a patient with right bundle branch block, persistent ST segment elevation, and ventricular fibrillation: a case report. Angiology. 1998;49:87-90.

  21. Veltmann C, Schimpf R, Echternach C, Eckardt L, Kuschyk J, Streitner F, et al. A prospective study on spontaneous fluctuations between diagnostic and non-diagnostic ECGs in Brugada syndrome: implications for correct phenotyping and risk stratification. Eur Heart J. 2006;27:2544-52.

  22. Hisamatsu K, Morita H, Fukushima KK, Takenaka S, Nagase S, Nakamura K, et al. Evaluation of the usefulness of recording the ECG in the 3rd intercostal space and prevalence of Brugada-type ECG in accordance with recently established electrocardiographic criteria. Circ J. 2004;68:135-8.

  23. 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.

  24. Antzelevitch C, Brugada P, Borggrefe M, Brugada J, Brugada R, Corrado D, et al. Brugada syndrome. Report of the second consensus conference. Circulation. 2005;111: 659-70.

  25. Richter S, Sarkozy A, Paparella G, Henkens S, Boussy T, Chierchia G, et al. Number of electrocardiogram leads displaying the diagnostic coved-type pattern in Brugada syndrome: a diagnostic consensus criterion to be revised. Eur Heart J. 2010;3:1357-64.

  26. Nakano Y, Shimizu W, Ogi1 H, Suenari K, Oda N, Makita Y, et al. A spontaneous type 1 electrocardiogram pattern in lead V2 is an independent predictor of ventricular fibrillation in Brugada syndrome. Europace. 2010;12:410-6.

  27. Tatsumi H, Tagaki M, Nagakawa E, Yamashita H, Yoshiyama M. Risk stratification in patients with Brugada syndrome: analysis of daily fluctuations in 12 lead electrocardiogram and signal-averaged electrocardiogram. J Cardiovasc Electrophysiol. 2006;17:705-11.

  28. Atarashi H, Ogawa S. New ECG criteria for hig-risk Brugada syndrome. Circ J. 2003;67:8-10.

  29. Castro J, Antzelevitch C, Tornés F, Dorantes S, Dorticós F, Zayas R, et al. Tpeak-end and Tpeak-end dispersion as risk factors for ventricular tachycardia/ventricular fibrillation in patients with the Brugada syndrome. J Am Coll Cardiol. 2006;47:182834.

  30. 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.

  31. Ohkubo K, Watanabe I, Okumura Y, Ashino S, Kofune M, Nagashima K, et al. Prolonged QRS duration in lead V2 and risk of life-threatening ventricular arrhythmia in patients with Brugada syndrome. Int Heart J. 2011;52:98-102.

  32. Morita H, Kusano KF, Miura D, Nagase S, Nakamura K, Morita ST, et al. Fragmented QRS as a marker of conduction abnormality and a predictor of prognosis of Brugada syndrome. Circulation. 2008;118:1697-1704.

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

  34. 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.

  35. Sarkozy A, Sorgente A, Boussy T, Casado R, Paparella G, Capulzini L, et al. The value of a family history of sudden death in patients with diagnostic type I Brugada ECG pattern. Eur Heart J. 2011;32:215360.

  36. Sacher F, Probst V, Iesaka Y, Jacon P, Laborderie J, Mizon-Gérard F, et al. Outcome after implantation of a cardioverter-defibrillator in patients with Brugada syndrome: A multicenter study. Circulation 2006; 114:2317-24.

  37. Sarkozy A, Boussy T, Kourgiannides G, Chierchia GB, Richter S, De Potter T, et al. Long-term follow-up of primary prophylactic implantable cardioverter-defibrillator therapy in Brugada syndrome. Eur Heart J. 2007;28:334-44.

  38. Tanaka H, Kinoshita O, Uchikawa S, Kasai H, Nakamura M, Izawa A, et al. Successful prevention of recurrent ventricular fibrillation by intravenous isoproterenol in a patient with Brugada syndrome. PACE. 2001;24:1293-4.

  39. Probst V, Evain S, Gournay V, Marie A, Schott JJ, Boisseau P, et al. Monomorphic ventricular tachycardia due to Brugada syndrome successfully treated by hydroquinidine therapy in a 3-year-old child. J Cardiovasc Electrophysiol. 2006;17:97-100.

  40. Paul M, Gerss J, Schulze-Bahr E, Wichter T, Vahlhaus C, Wilde AM, et al. Role of programmed ventricular stimulation in patients with Brugada syndrome: a meta-analysis of worldwide published data. Eur Heart J. 2007;28:2126-33.

  41. Brugada P. Amid the fourth lustrum after the description of Brugada syndrome: controversies over? Europace. 2009;11:412-3.

  42. Viskin S, Rogowski O. Asymptomatic Brugada syndrome: a cardiac ticking time-bomb? Europace. 2007;9:707-10.

  43. Viskin S, Rosso R. Risk of sudden death in asymptomatic Brugada syndrome. Not as high as we thought and not as low as we wished…but the contrary. J Am Coll Cardiol. 2010;56:1585-8.

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

  45. Belhassen B, Viskin S, Fish R, Glick A, Setbon I, Eldar M. Effects of electrophysiologic guided therapy with Class IA antiarrhythmic drugs on the long-term outcome of patients with idiopathic ventricular fibrillation with or without the Brugada syndrome. J Cardiovasc Electrophysiol. 1999;10:1301-12.

  46. Haissaguerre M, Extramiana F, Hocini M, Cauchemez B, Jaïs P, Cabrera JA, et al. Mapping and ablation of ventricular fibrillation associated with long-QT and Brugada syndromes. Circulation. 2003;108:925-8.




2020     |     www.medigraphic.com