avkАрхивъ внутренней медициныThe Russian Archives of Internal Medicine2226-67042411-6564“SINAPS” LLC10.20514/2226-6704-2016-6-2-61-69avk-521Research ArticleРАЗБОР КЛИНИЧЕСКИХ СЛУЧАЕВANALYSIS OF CLINICAL CASESСИНДРОМ БРУГАДА: ОТ ПЕРВИЧНОЙ ЭЛЕКТРИЧЕСКОЙ БОЛЕЗНИ СЕРДЦА К МОРФОЛОГИЧЕСКОМУ СУБСТРАТУBRUGADA SYNDROME: FROM PRIMARY ELECTRIC HEART DISEASE TO MORPHOLOGICAL SUBSTRATEПавленкоТ. А.PavlenkoT. A.

6 курс, лечебный факультет, 

г. Москва

6 year, General Medicine,

Moscow

pavlenko1307@mail.ruБлаговаО. В.BlagovaO. V.

кафедра факультетской терапии № 1,

г. Москва

Chair of Internal Medicine № 1,

Moscow

Первый Московский государственный медицинский университет им. И.М. СеченоваРоссияFirst Moscow State Medical University named after I.M. SechenovRussian Federation201604052016626169Copyright © Павленко Т.А., Благова О.В., 20162016Павленко Т.А., Благова О.В.Pavlenko T.A., Blagova O.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.medarhive.ru/jour/article/view/521

Рост интереса к каналопатиям и развитие их диагностики делают синдром Бругада все более актуальной проблемой для врачей. Ранее популярная концепция «первичной электрической болезни сердца» не может объяснить отсутствие единого генетического субстрата происхождения синдрома, его частое сочетание с другими нарушениями ритма и наличие фенокопий. Особый интерес представляют сочетания с. Бругада с морфологическими изменениями миокарда, в том числе с миокардитом. Именно воспаление ассоциируется с клинической манифестацией синдрома, что заставляет задуматься о первичности данной каналопатии и искать другие возможные механизмы аритмогенеза с. Бругада.

Nowadays interest in channelopathies is growing, and developing diagnostic capabilities make Brugada syndrome much more actual problem than ever. Conception of «primary electric heart disease», which earlier was so popular, now couldn’t explain the absence of single genetic syndromes’ substrate, its’ common combinations with another arrhythmias and the presence of Brugada phenocopy. Conjunction of Brugada syndrome with morphological changes in myocardium , especially myocarditis, is an area of special interest. Infl ammation associates with clinical manifestation of the syndrome, and this situation forces to think about channelopathies’ primacy and to look for another possible mechanisms of Brugada syndrome.

синдром Бругадамиокардитканалопатияфенокопияэлектрическое ремоделированиеBrugada syndromemyocarditischannelopathiesphenocopyelectric remodellingReferencesБокерия Л. А., Бокерия О. Л., Киртбая Л. Н. Синдром Бругада: клеточные механизмы и подходы к лечению. Анналы аритмологии 2010. 7, 3: p. 16-30.Bockeria L. A., Bockeria O. L., Kirtbaya L. N. Brugada syndrome: cellular mechanisms and approaches to treatment. Annaly aritmologii 2010. 7, 3: p. 16-30.De Luna A.B., MD, PhD, Brugada J., MD, PhD, Baranchuk A., MD, Borggrefe M., MD, Guenter Breithardt, MD, Goldwasser D., MD, Lambiase P., MD, Riera A.P., MD, PhD, Garcia-Niebla J., RN, Pastore C., MD, PhD, Oreto G., MD, McKenna W., MD, Zareba W., MD, PhD, Brugada R., MD, PhD, Brugada P., MD, PhD. Current electrocardiographic criteria for diagnosis of Brugada pattern: a consensus report. Journal of Electrocardiology 2012. 45: 433–442.De Luna A.B., MD, PhD, Brugada J., MD, PhD, Baranchuk A., MD, Borggrefe M., MD, Guenter Breithardt, MD, Goldwasser D., MD, Lambiase P., MD, Riera A.P., MD, PhD, Garcia-Niebla J., RN, Pastore C., MD, PhD, Oreto G., MD, McKenna W., MD, Zareba W., MD, PhD, Brugada R., MD, PhD, Brugada P., MD, PhD. Current electrocardiographic criteria for diagnosis of Brugada pattern: a consensus report. Journal of Electrocardiology 2012. 45: 433–442.Yan G.X., Antzelevitch C. Cellular basis for the Brugada syndrome andother mechanisms of arrhythmogenesis associated with STsegmentelevation. Circulation 1999. 100: 166.Yan G.X., Antzelevitch C. Cellular basis for the Brugada syndrome andother mechanisms of arrhythmogenesis associated with ST-segmentelevation. Circulation 1999. 100:166.Aiba T., MD, PHD, Shimizu W., MD, PHD, Hidaka I., MS, Uemura K., MD, Noda T., MD, PHD, Zheng C., PHD, Kamiya A., MD, Inagaki M., MD, Sugimachi M., MD, PHD, Sunagawa K., MD, PHD. Cellular Basis for Trigger and Maintenance of Ventricular Fibrillation in the Brugada Syndrome Model. High-Resolution Optical Mapping Study. Journal of the American College of Cardiology 2006. 47, 10: 2074–2085.Aiba T., MD, PHD, Shimizu W., MD, PHD, Hidaka I., MS, Uemura K., MD, Noda T., MD, PHD, Zheng C., PHD, Kamiya A., MD, Inagaki M., MD, Sugimachi M., MD, PHD, Sunagawa K., MD, PHD. Cellular Basis for Trigger and Maintenance of Ventricular Fibrillation in the Brugada Syndrome Model. High-Resolution Optical Mapping Study. Journal of the American College of Cardiology 2006. 47, 10: 2074–2085.Kimura M., MD, Kobayashi T., MD, Owada S., MD, Ashikaga K., MD, Higuma T., MD, Sasaki S., MD, Iwasa A., MD, Motomura S., MD, Okumura K., MD. Mechanism of ST Elevation and Ventricular Arrhythmias in an Experimental Brugada Syndrome Model. Circulation 2004. 109: 125-131.Kimura M., MD, Kobayashi T., MD, Owada S., MD, Ashikaga K., MD, Higuma T., MD, Sasaki S., MD, Iwasa A., MD, Motomura S., MD, Okumura K., MD. Mechanism of ST Elevation and Ventricular Arrhythmias in an Experimental Brugada Syndrome Model. Circulation 2004. 109:125-131.Antzelevitch C., PhD, FAHA. M Cells in the Human Heart. Circ Res 2010. 106(5): 815–817.Antzelevitch C., PhD, FAHA. M Cells in the Human Heart. Circ Res 2010. 106(5): 815–817.Hoogendijk M.G., Potse M., Linnenbank A.C., et al. Mechanism of right precordial ST-segment elevation in structural heart disease: excitation failure by current-to-load mismatch. Heart Rhythm 2010. 7: 238.Hoogendijk M.G., Potse M., Linnenbank A.C., et al. Mechanism of right precordial ST-segment elevation in structural heart disease: excitation failure by current-to-load mismatch. Heart Rhythm 2010. 7:238.Peréz-Riera A.R., Ferreira Filho C., de Abreu L.C., et al; On behalf of the International VCG Investigators Group. Do patients with electrocardiographic Brugada type 1 pattern have associated right bundle branch block? A comparative vectorcardiographic study. Europace 2012. Jan 10. [Epub ahead of print].Peréz-Riera A.R., Ferreira Filho C., de Abreu L.C., et al; On behalf of the International VCG Investigators Group. Do patients with electrocardiographic Brugada type 1 pattern have associated right bundle branch block? A comparative vectorcardiographic study. Europace 2012. Jan 10. [Epub ahead of print].Anselm D.D., Evans J.M., Baranchuk A. Brugada phenocopy: A new electrocardiogram phenomenon. World J Cardiol. 2014 Mar 26; 6(3): 81–86.Anselm D.D., Evans J.M., Baranchuk A. Brugada phenocopy: A new electrocardiogram phenomenon. World J Cardiol. 2014 Mar 26; 6(3): 81–86.Gottschalk B.H., Anselm D.D., Brugada J., Brugada P., Wilde A.A., Chiale P.A., Pérez-Riera A.R., Elizari M.V., de Luna A.B., Krahn A.D., Tan H.L., Postema P.G., Baranchuk A. Expert cardiologists cannot distinguish between Brugada phenocopy and Brugada syndrome electrocardiogram patterns. Europace 2015 Oct 25. [Epub ahead of print].Gottschalk B.H., Anselm D.D., Brugada J., Brugada P., Wilde A.A., Chiale P.A., Pérez-Riera A.R., Elizari M.V., de Luna A.B., Krahn A.D., Tan H.L., Postema P.G., Baranchuk A. Expert cardiologists cannot distinguish between Brugada phenocopy and Brugada syndrome electrocardiogram patterns. Europace 2015 Oct 25. [Epub ahead of print].Anselm D.D., Baranchuk A. Brugada Phenocopy Emerging as a New Concept. Rev Esp Cardiol. 2013; 66: 755.Anselm D.D., Baranchuk A. Brugada Phenocopy Emerging as a New Concept. Rev Esp Cardiol. 2013;66:755.Rodrigues R., Amador P., Rassi L., et al. Padrão de Brugada em doente medicada com lamotrigina. Rev PortCardiol. 2013;32:807-810. 13. Genaro N.R., Anselm D.D., Cervino N., Estevez A.O., Perona C., Villamil A.M., et al. Brugada Phenocopy Clinical Reproducibility Demonstrated by Recurrent Hypokalemia. Annals of Noninvasive Electrocardiology 2014 19,4: 387–390.Rodrigues R., Amador P., Rassi L., et al. Padrão de Brugada em doente medicada com lamotrigina. Rev PortCardiol. 2013;32:807-810.Frustaci A., MD, Priori S.G., MD, PhD, Pieroni M., MD, PhD, Chimenti C., MD, PhD, Napolitano C., MD, PhD, Rivolta I., PhD, Sanna T., MD, Bellocci F., MD, Russo M.A., MD. Cardiac Histological Substrate in Patients With Clinical Phenotype of Brugada Syndrome. Circulation 2005. 112: 3680-3687.Genaro N.R., Anselm D.D., Cervino N., Estevez A.O., Perona C., Villamil A.M., et al. Brugada Phenocopy Clinical Reproducibility Demonstrated by Recurrent Hypokalemia. Annals of Noninvasive Electrocardiology 2014 19,4: 387–390.Bezzina C.R., Rook M.B., Groenewegen A., Herfst L.J., van der Wal A.C., Lam J., Jongsma H.J., Wilde A.A.M., Mannens M.M. Compound heterozygosity for mutations (W156X and R225W) in SCN5A associated with severe cardiac conduction disturbances and degenerative changes in the conduction system. Circ Res 2003. 92: 159–168.Frustaci A., MD, Priori S.G., MD, PhD, Pieroni M., MD, PhD, Chimenti C., MD, PhD, Napolitano C., MD, PhD, Rivolta I., PhD, Sanna T., MD, Bellocci F., MD, Russo M.A., MD. Cardiac Histological Substrate in Patients With Clinical Phenotype of Brugada Syndrome. Circulation 2005. 112:3680-3687.Bonny A., Tonet J., Marquez M.F., De Sisti A., Temfemo A., Himbert C., Gueffaf F., Larrazet F., Ditah I., Frank R., Hidden-Lucet F., Fontaine G. C-Reactive Protein Levels in the Brugada Syndrome. Cardiol Res Pract. 2011; 2011: 341521.Bezzina C.R., Rook M.B., Groenewegen A., Herfst L.J., van der Wal A.C., Lam J., Jongsma H.J., Wilde A.A.M., Mannens M.M. Compound heterozygosity for mutations (W156X and R225W) in SCN5A associated with severe cardiac conduction disturbances and degenerative changes in the conduction system. Circ Res 2003. 92:159 –168.Juhasz Z., Tiszlavicz L., Kele B., Terhes G., Deak J., Rudas L., Kereszty E. Sudden cardiac death from parvovirus B19 myocarditis in a young man with Brugada syndrome. J Forensic Leg Med. 2014 Jul; 25: 8-13.Bonny A., Tonet J., Marquez M.F., De Sisti A., Temfemo A., Himbert C., Gueffaf F., Larrazet F., Ditah I., Frank R., Hidden-Lucet F., Fontaine G. C-Reactive Protein Levels in the Brugada Syndrome. Cardiol Res Pract. 2011; 2011: 341521.Buob A., Siaplaouras S., Janzen I., Schwaab B., Hammer B., Schneider G., Kandolf R., Böhm M., Jung J. Focal parvovirus B19 myocarditis in a patient with Brugada syndrome. Cardiol Rev. 2003 Jan-Feb; 11(1): 45-9.Juhasz Z., Tiszlavicz L., Kele B., Terhes G., Deak J., Rudas L., Kereszty E. Sudden cardiac death from parvovirus B19 myocarditis in a young man with Brugada syndrome. J Forensic Leg Med. 2014 Jul;25:8-13.Chimenti C., Russo M.A., Frustaci A., Verardo R., Caldarulo M., Acconcia M.C. Myocarditis in hypertrophic cardiomyopathy patients presenting acute clinical deterioration. Eur Heart J 2007 28 (6): 733-740.Buob A., Siaplaouras S., Janzen I., Schwaab B., Hammer B., Schneider G., Kandolf R., Böhm M., Jung J. Focal parvovirus B19 myocarditis in a patient with Brugada syndrome. Cardiol Rev. 2003 Jan-Feb;11(1):45-9.Salerno F., Girerd N., Chalabreysse L., Billaud G., Lina B., Chevalier P. Myocarditis and cardiac channelopathies: A deadly association? International Journal of Cardiology 2011. 147, 3: 468 — 470.Chimenti C., Russo M.A., Frustaci A., Verardo R., Caldarulo M., Acconcia M.C. Myocarditis in hypertrophic cardiomyopathy patients presenting acute clinical deterioration. Eur Heart J 2007 28 (6): 733-740.Patane S., Marte F., La Rosa F.C., et al. Revelation of Brugada electrocardiographic pattern during a febrile state. Int J Cardiol 2010 Apr 1; 140(1): 19–21.Salerno F., Girerd N., Chalabreysse L., Billaud G., Lina B., Chevalier P. Myocarditis and cardiac channelopathies: A deadly association? International Journal of Cardiology 2011. 147, 3: 468 – 470.Chevalier P., FESC, Scridon A. Ventricular arrhythmias complicating acute myocarditis. E-journal of the ESC Council for Cardiology Practice Apr 2011. Vol 9, N° 26.Patane S., Marte F., La Rosa F.C., et al. Revelation of Brugada electrocardiographic pattern during a febrile state. Int J Cardiol 2010 Apr 1;140(1): 19–21.Wakisaka Y., Niwano S., Niwano H., Saito J., Yoshida T., Hirasawa S., Kawada H., Izumi T. Structural and electrical ventricular remodeling in rat acute myocarditis and subsequent heart failure. Cardiovascular Research 2004. 63 (4): 689-699.Chevalier P., FESC, Scridon A. Ventricular arrhythmias complicating acute myocarditis. E-journal of the ESC Council for Cardiology Practice Apr 2011. Vol 9, N° 26.Park H., Park H., Lim J., Oh S., Park S., MD, PhD, Pak H., MD, PhD, Lee M., MD, PhD, Joung B., MD, PhD. Arrhythmogenic Mechanisms of Autoimmune Myocarditis Associated with Inflammation and Ca2+/Calmodulin- Dependent Protein Kinase II Activation. Int J Arrhythm. 2014. 15 (2): 4-16.Wakisaka Y., Niwano S., Niwano H., Saito J., Yoshida T., Hirasawa S., Kawada H., Izumi T. Structural and electrical ventricular remodeling in rat acute myocarditis and subsequent heart failure. Cardiovascular Research 2004. 63 (4): 689-699.Caforio A.L., Pankuweit S., Arbustini E., Basso C., Gimeno-Blanes J., Felix S.B., Fu M., Heliö T., Heymans S., Jahns R., Klingel K., Linhart A., Maisch B., McKenna W., Mogensen J., Pinto Y.M., Ristic A., Schultheiss H.P., Seggewiss H., Tavazzi L., Thiene G., Yilmaz A., Charron P., Elliott P.M. Current state of knowledge on aetiology, diagnosis, management, and therapy of myocarditis: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2013 Sep. 34(33): 2636-48, 2648a-2648d.Park H., Park H., Lim J., Oh S., Park S., MD, PhD, Pak H., MD, PhD, Lee M., MD, PhD, Joung B., MD, PhD. Arrhythmogenic Mechanisms of Autoimmune Myocarditis Associated with Inflammation and Ca2+/Calmodulin- Dependent Protein Kinase II Activation. Int J Arrhythm. 2014. 15 (2): 4-16.Благова О.В., Осипова Ю.В., Недоступ А.В., Коган Е.А. Неинвазивные диагностические критерии миокардита в сопоставлении с результатами морфологического исследования. Российский национальный конгресс кардиологов, Москва, 23 сентября 2015 г.Caforio A.L., Pankuweit S., Arbustini E., Basso C., Gimeno-Blanes J., Felix S.B., Fu M., Heliö T., Heymans S., Jahns R., Klingel K., Linhart A., Maisch B., McKenna W., Mogensen J., Pinto Y.M., Ristic A., Schultheiss H.P., Seggewiss H., Tavazzi L., Thiene G., Yilmaz A., Charron P., Elliott P.M. Current state of knowledge on aetiology, diagnosis, management, and therapy of myocarditis: a position statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Diseases. Eur Heart J. 2013 Sep. 34(33):2636-48, 2648a-2648d.Francis J., MD, DM, FCSI, FACC and Antzelevitch C., PhD, FACC, FAHA, FHRS. Atrial Fibrillation and Brugada Syndrome. J Am Coll Cardiol. 2008. Mar 25; 51(12): 1149–1153.Blagova O.V., Osipova Y.V., Nedostup A.V., Kogan E.A. Non-invasive diagnostic criteria of myocarditis comparing to results of morphological tests. Russian National Cardiology Congress, Moscow, 2015, Sept. 23.Francis J., MD, DM, FCSI, FACC and Antzelevitch C., PhD, FACC, FAHA, FHRS. Atrial Fibrillation and Brugada Syndrome. J Am Coll Cardiol. 2008. Mar 25; 51(12): 1149–1153.Francis J., MD, DM, FCSI, FACC and Antzelevitch C., PhD, FACC, FAHA, FHRS. Atrial Fibrillation and Brugada Syndrome. J Am Coll Cardiol. 2008. Mar 25; 51(12): 1149–1153.

The authors declare that there are no conflicts of interest present.