S.D. MAYANSKAYA¹, N.А.TSYBULKIN²

¹Kazan State Medical University, 49 Butlerov St., Кazan, Russian Federation, 420012

²Kazan State Medical Academy, 36 Butlerov St., Кazan, Russian Federation, 420012

Brugada syndrome  

Маyanskaya S.D. — D. Med. Sc., Professor, Department of hospital therapy with coarse of endocrinology, tel. +7-905-316-99-66, e-mail: Smayanskaya@mail.ru

Tsybulkin N.A. — Cand. Med. Sc., Associate Professor of the Department of functional diagnostics, tel. (843) 261-74-11, e-mail: diagnost@ksu.ru

This review consideres the etiological factors and electrophysiological development mechanisms of this disease. Special attention is paid to the diagnosis of Brugada syndrome using the methods available in daily cardiology practice. There is presented modern management of patients with this disease, the possibility of drug prevention and the use of implantable cardioverter-defibrillators, as well as certain long-term prognosis in these patients.

Key words: Brugada syndrome, diagnostics, treatment, prevention.

REFERENCES

1. http://cardioplaneta.ru/expert/cardiology/arrhythmia/58-sindrom-brugada.html
2. 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, vol. 20, pp. 1391-1396.
3. Bokeria L.A., Boqueria O.L., Kirtbaya L.N. Brugada syndrome: cellular mechanisms and approaches to the treatment. Annals aritmologii, 2010, vol. 3, pp. 16-31.
4. Donohue D., Tehrani F., Jamehdor R., Lam C., Movahed M.R. The prevalence of Brugada ECG in adult patients in a large university hospital in the Western United States. Am Heart Hosp J, 2008, vol. 6, pp. 48-50.
5. Antzelevitch C., Brugada P., Borggrefe M. et al. Brugada syndrome: Report of the Second Consensus Conference: Endorsed by the Heart Rhythm Society and the European Heart Rhythm Association. Circulation, 2005, vol. 111, pp. 659-670.
6. Shimuzu W., Matsuo K., Kokubo Y. et al. Sex Hormone and gender difference – Role of testosterone on male preponderance in Brugada Syndrome. J Cardiovasc Electrophysiol, 2007, vol. 18, pp. 415-421.
7. Miyasaka Y., Tsuji H., Yamada K. et al. Prevalence and mortality of the Brugada-type electrocardiogram in one city in Japan. J Am Coll Cardiol, 2001, vol. 38, pp. 771-774.
8. Hermida J.S., Lemoine J.L., Aoun F.B. et al. Prevalence of the Brugada syndrome in an apparently healthy population. Am J Cardiol, 2000, vol. 86, pp. 91-94.
9. Chen Q., Kirsch G.E., Zhang D. et al. Genetic basis and molecular mechanism for idiopathic ventricular fibrillation. Nature, 1998, vol. 392, pp. 293-296.
10. Rook M.B., Bezzina Alshinawi C., Groenewegen W.A. et al. Human SCN5A gene mutations alter cardiac sodium channel kinetics and are associated with the Brugada syndrome. Cardiovasc. Res, 1999, vol. 44, pp. 507-517.
11. Deschênes I., Baroudi G., Berthet M. et al. Electrophysiological characterization of SCN5A mutations causing long QT(E1784K) and Brugada (R1512W and R1432G) syndromes. Cardiovasc. Res, 2000, vol. 46, pp. 55-65.
12. Napolitano C., Priori S.G. Brugada syndrome. Orphanet journal of rare diseases, 2006, vol. 1, p. 35.
13. Vatta M., Dumaine R., Antzelevitch C. et al. Novel mutations in domain I of SCN5A cause Brugada syndrome. Mol Genet Metab, 2002, vol. 75, p. 317-324.
14. Cordeiro J.M., Barajas-Martinez H., Hong K. et al. Compound heterozygous mutations P336L and I1660V in the human cardiac sodium channel associated with the Brugada syndrome. Circulation, 2006, vol. 114, pp. 2026-2033.
15. Casini S., Tan H.L, Bhuiyan Z.A. et al. Characterization of a novel SCN5A mutation associated with Brugada syndrome reveals involvement of DIIIS4-S5 linker in slow inactivation. Cardiovasc. Res, 2007, vol. 76, rr. 418-429.
16. Antzelevitch C, Pillevick GD, Cordeiro JM, 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, vol. 115. pp.442-449.
17. Makita N., Behr E., Shimizu W. et al. The E1784K mutation in SCN5A is associated with mixed clinical phenotype of type 3 long Q-T syndrome. J. Clin. Invest., 2008, vol. 118, pp. 2219-2229.
18. Moiseyev V.S., Kiyakbaev G.K. Brugada syndrome. Cardiomyopathy and myocarditis. Moscow: GEOTAR Media, 2011. Pp. 119-128.
19. Hong K., Brugada J., Oliva A. et al. Value of electrocardiographic parameters and ajmaline test in the diagnosis of Brugada syndrome caused by SCN5A mutations. Circulation, 2004, vol. 110, pp. 3023-3207.
20. London B., Michalec M., Mehdi H. et al. Mutation in glycerol-3-phosphate dehydrogenase 1-like gene (GPD1-L) decreases cardiac Na+ current and causes inherited arrhythmias. Circulation, 2007, vol. 116, rr. 2260-2268.
21. Hong K., Guerchicoff A., Pollevick G.D. et al. Cryptic 5′ splice site activation in SCN5A associated with Brugada syndrome. J. Mol. Cell. Cardiol., 2005, vol. 38, pp. 555-560.
22. Kaufman E.S. Genetic Testing in Brugada Syndrome. J. the Amer. Coll. of Cardiol. 2012, vol. 60, r. 15.
23. Weiss R., Barmada M.M., Nguyen T. et al. Clinical and molecular heterogeneity in the Brugada syndrome: A novel gene locus on chromosome 3. Circulation, 2002, vol. 105, r. 707-713.
24. Yan G.X., Antzelevitch C. Cellular basis for the Brugada syndrome and other mechanisms of arrhythmogenesis associated with ST-segment elevation. Circulation, 1999, vol. 100, rr. 1660-1666.
25. Skinner J.R., Chung S.K., Nel C.A. et al. Brugada Syndrome masquerading as febrile seizures. J. Pediatrics, 2007, vol. 119, pp. 1206-1211.
26. Wilde A.A.M., Antzelevitch C., Borggrefe M. et al. Proposed diagnostic criteria for the Brugada syndrome. Eur Heart J., 2002, vol. 23, pp. 1648-1654.
27. Priori S.G., Napolitano C., Schwartz P.J. Brugada Syndrome.In Braunwald’s Heart Disease: A Textbook of Cardiovascular Medicine. 8th ed. Saunders: An Imprint of Elsevier, 2008. Pp. 105-106.
28. Makarov L.M. Brugada Syndrome. Medical emergency conditions, 2008, vol. 1(14), pp. 96-99.
29. 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, vol. 101, rr. 510-515.
30. Tokioka K., Kusano K.F., Morita H. et al. Electrocardiographic parameters and fatal arrhythmic events in patients with Brugada syndrome: combination of depolarization and repolarization abnormalities. J. Am. Coll. Cardiol., 2014, vol. 63, no. 20, pp. 2131-2138.
31. Conte G., Dewals W., Sieira J. et al. Drug-induced brugada syndrome in children: clinical features, device-based management, and long-term follow-up. J. Am. Coll. Cardiol., vol. 63(21), pp. 2272-9.
32. Morito H., Kusano K.F., Minra D. et al. Fragmented QRS as a marker of conduction abnormality and a predictor of prognosis of Brugada Syndrome. Circulation, 2008, vol. 118, rr. 1697-1704.
33. Francis J Antzelevitch C. Atrial Fibrillation and Brugada Syndrome. J Am Coll Cardiol, 2008, vol. 51, pp.1149-1153.
34. Furukawa Y., Akahane K., Ogiwara Y., Chiba S. K+-channel blocking and anti-muscarinic effects of a novel piperazine derivative, INO 2628, on the isolated dog atrium. Eur. J. Pharm., 1991, vol. 193, pp. 217-222.
35. Cortez-Dias N., Plácido R., Marta L. et al. Epicardial ablation for prevention of ventricular fibrillation in a patient with Brugada syndrome. Rev Port Cardiol., 2014, vol. 33, no. 5b–305 e1-7.
36. Probst V., Veltman L., Eckardt P.G. et al. Long-term prognosis of patients diagnosed with Brugada Syndrome. Results from the FINGER Brugada Syndrome Registry. Circulation, 2010, vol. 121, pp. 633-643.
37. Priori S.G., Napolitano C. Management of patients with Brugada Syndrome should not be based on programmed stimulation. Circulation, 2005, vol. 112, pp. 285-291.
38. Gehi A.K., Duong T.D., Metz L.D. et al. Risk stratification of individuals with the Brugada electrocardiogram: A meta analysis. J. Cardiovasc Electrophysiol, 2006, vol. 17, pp. 577-583.
39. Brugada J., Brugada R., Brugada P. Right bundlebranch block and ST-segment elevation in leads V1 through V3. A marker for sudden death in patients without demonstrable structural heart disease. Circulation, 1998, vol. 7, pp. 457-460.
40. Belhassen B., Glick A., Viskin S. Efficacy of quinidine in high-risk patients with Brugada syndrome. Circulation, 2004, vol. 110(13), pp. 1731-1737.
41. Conte G., Sieira J., Ciconte G. et al. Implantable Cardioverter-Defibrillator. Therapy in Brugada Syndrome A 20-Year Single-Center Experience. J. Amer. Coll. Card, 2015, vol. 65, vol. 9, pp. 879-888.
42. Mashar M., Kwok A.J., Pinder R. et al. The Brugada syndrome revisited. Trends in Cardiovascular Medicine, 2014, vol. 24, rr. 191-196.
43. Postema P.G., Wolpert C., Amin A.S. et al. Drugs and Brugada Syndrome patients: Review of the literature, recommendations and an up-to-date, available at: www.brugadadrugs.org, Heart Rhythm., 2009, vol. 6, pp. 1335-1341.
44. Babaliaros V.C., Hurst J.W. Tricyclic antidepressants and the Brugada syndrome: an example of Brugada waves appearing after the administration of desipramine. Clin. Cardiol, 2002, vol. 25, pp. 395-398.
45. Brugada J., Brugada R. Brugada syndrome. Arch. Mal. Coeur. Vaiss, 1999, vol. 92, pp. 847-850.
46. Tsuchiya T., Ashikaga K., Honda T. et al. Prevention of ventricular fibrillation by cilostazol, an oral phosphodiesterase inhibitor, in a patient with Brugada syndrome. J. Cardiovasc. Electrophysiol, 2002, vol. 13(7), rr. 698-701.
47. Szél T., Koncz I., Antzelevitch C. Cellular mechanisms underlying the effects of milrinone and cilostazol to suppress arrhythmogenesis associated with Brugada syndrome. Heart Rhythm., 2013, vol. 10(11), pp. 1720-1727.
48. Brugada P., Brugada R., Campuzano O. Brugada Syndrome 1992-2012: Twenty Years of Scientific Progress. Cardiac Electrophysiology: From Cell to Bedside, 2012, vol. 92, rr. 925-933.
49. Antzelevitch C., Brugada R. Guest Editorial: Fever and Brugada Syndrome. PACE, 2002, vol. 25, pp. 1537-1539.