zdorПроблемы здоровья и экологииHealth and Ecology Issues2220-09672708-6011Gomel State Medical University10.51523/2708-6011.2009-6-4-22zdor-896Research ArticleКЛИНИЧЕСКАЯ МЕДИЦИНАCLINICAL MEDICINEСиндром удлиненного интервала QT у детейLONG QT SYNDROM IN CHILDRENЛашковскаяТ. А.LashkovskajaT. A.noemail@neicon.ruКарчевскийА. А.KarcheuskiA. A.noemail@neicon.ruГродненский государственный медицинский университетGrodno State Medical University20092812200904111115Copyright © Лашковская Т.А., Карчевский А.А., 20092009Лашковская Т.А., Карчевский А.А.Lashkovskaja T.A., Karcheuski A.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.gsmu.by/jour/article/view/896

В статье представлен обзор современной литературы по проблеме этиологии, классификации, патогенезу и основным клиническим проявлениям синдрома удлиненного интервала QT у детей. Даны электрокардиографические критерии диагностики синдрома, особенности синкопальных состояний, возможные пути профилактики жизнеугрожающих аритмий у детей.

In the article, there is a survey of modern literature as for problem of etiology, classification, pathogenesis and major clinical manifestations of long QT syndrome in children. The electrocardiographyc criteria for diagnosis of the syndrome, features of syncopic states and the possible ways of life-threatening arrhythmias prophylaxis in children are discussed.

детисиндром удлиненного интервала QTсинкопеэтиологияклассификациядиагностикапрофилактикалечениеchildrenlong QT syndromesyncopeetiologyclassificationclassificationdiagnosticspreventiontreatmentReferencesШкольникова, М. А. Жизнеугрожающие аритмии у детей / М. А. Школьникова. - М.: Нефтяник, 1999. - 230 с.Школьникова, М. А. Жизнеугрожающие аритмии у детей / М. А. Школьникова. - М.: Нефтяник, 1999. - 230 с.Redusing mortality from sudden cardiac death in the communty: lessons from epidemiology and clinical applications research / N. Sotoodehnia [et al.] // Cardiovasc Res. - 2000. - Vol. 50. - P. 197-209.Redusing mortality from sudden cardiac death in the communty: lessons from epidemiology and clinical applications research / N. Sotoodehnia [et al.] // Cardiovasc Res. - 2000. - Vol. 50. - P. 197-209.Sudden cardiac death in young people with apparently normal heart / D. Corrado [et al.] // Cardiovasc Res. - 2001. - Vol. 50(2). - P. 399-408.Sudden cardiac death in young people with apparently normal heart / D. Corrado [et al.] // Cardiovasc Res. - 2001. - Vol. 50(2). - P. 399-408.Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval and sudden death / A. Jervella [et al.] // Am Heart J. - 1957. - Vol. 54. - P. 59-68.Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval and sudden death / A. Jervella [et al.] // Am Heart J. - 1957. - Vol. 54. - P. 59-68.The long QT syndrome: prospective longitudinal study of 328 families / A. Moss [et al.] // Circul. - 1991. - Vol. 84. - P. 1136-1144.The long QT syndrome: prospective longitudinal study of 328 families / A. Moss [et al.] // Circul. - 1991. - Vol. 84. - P. 1136-1144.A famili histori of seizures associated with sudden cardiac deaths / J. T. Bricker [et al.] // Am. J Dis Child. - 1984. - Vol. 138. - P. 866-868.A famili histori of seizures associated with sudden cardiac deaths / J. T. Bricker [et al.] // Am. J Dis Child. - 1984. - Vol. 138. - P. 866-868.Antzelevitch, C. Molecular genetics of arrhythmias and cardiovascular conditions associated with arrhythmias. From the Masonic Medical Research Laboratory / C. Antzelevitch // Utica, N.Y. PACE. - 2003. - Vol. 26. - P. 2194-2208.Antzelevitch, C. Molecular genetics of arrhythmias and cardiovascular conditions associated with arrhythmias. From the Masonic Medical Research Laboratory / C. Antzelevitch // Utica, N.Y. PACE. - 2003. - Vol. 26. - P. 2194-2208.Vincent, G. M. Long QT syndrome / G. M. Vincent // Cardiol. Clin. - 2000. - Vol. 18. - P. 309-325.Vincent, G. M. Long QT syndrome / G. M. Vincent // Cardiol. Clin. - 2000. - Vol. 18. - P. 309-325.Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2 / I. Splawski [et al.] // Circulation. - 2000. - Vol. 102. - P. 1178-1185.Spectrum of mutations in long-QT syndrome genes. KVLQT1, HERG, SCN5A, KCNE1, and KCNE2 / I. Splawski [et al.] // Circulation. - 2000. - Vol. 102. - P. 1178-1185.Ca(V) 1.2 calcium channel dysfunction causes a multisystem disorder including arrhythmia and autism / I. Splawski [et al.] // Cell. - 2004. - Vol. 19. - P. 19-31.Ca(V) 1.2 calcium channel dysfunction causes a multisystem disorder including arrhythmia and autism / I. Splawski [et al.] // Cell. - 2004. - Vol. 19. - P. 19-31.Genomic structure of three long QT syndrome genes: KVLQTI, HERG, and KCNEI / I. Splawski [et al.] // Genomics. - 1998. - Vol. 51. - P. 86-97.Genomic structure of three long QT syndrome genes: KVLQTI, HERG, and KCNEI / I. Splawski [et al.] // Genomics. - 1998. - Vol. 51. - P. 86-97.Molecular genetic analysis of long QT syndrome in 67 Russian families / E. Zaklyazminskaya [et al.] // Eur. Heart J. - 2003. - Vol. 24. - P. 44.Molecular genetic analysis of long QT syndrome in 67 Russian families / E. Zaklyazminskaya [et al.] // Eur. Heart J. - 2003. - Vol. 24. - P. 44.Genetic and molecular basic of cardiac arrhythmias. Impact on clinical management / J. Barhanin [et al.] // Eur. Heart J. - 1999. - Vol. 20. - P. 174-195.Genetic and molecular basic of cardiac arrhythmias. Impact on clinical management / J. Barhanin [et al.] // Eur. Heart J. - 1999. - Vol. 20. - P. 174-195.Cellular basis for long QT, transmural dispersion of repolarization and torsade de pointes in the long QT syndrome / W. Shimizu [et al.] // J. Electrocardiol. - 1999. - Vol. 32. - P. 177-184.Cellular basis for long QT, transmural dispersion of repolarization and torsade de pointes in the long QT syndrome / W. Shimizu [et al.] // J. Electrocardiol. - 1999. - Vol. 32. - P. 177-184.Epinephrine-induced QT interval prolongation: A genespecific paradoxical response in congenital long QT syndrome / М. J. Ackerman [et al.] // Mayo Clin. Proc. - 2002. - Vol. 77. - P. 413-421.Epinephrine-induced QT interval prolongation: A genespecific paradoxical response in congenital long QT syndrome / М. J. Ackerman [et al.] // Mayo Clin. Proc. - 2002. - Vol. 77. - P. 413-421.Case Evaluation and treatment of pediatric patients with congenital or acquired long QT interval syndromes / J. R. Phillips [et al.] // Progress in Pediatric Cardiology. - 2001. - Vol. 13. - P. 101-110.Case Evaluation and treatment of pediatric patients with congenital or acquired long QT interval syndromes / J. R. Phillips [et al.] // Progress in Pediatric Cardiology. - 2001. - Vol. 13. - P. 101-110.Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias / P. J. Schwartz [et al.] // Circulation. - 2001. - Vol. 103(1). - P. 89-95.Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias / P. J. Schwartz [et al.] // Circulation. - 2001. - Vol. 103(1). - P. 89-95.Vincent, G. M. The molecular genetics of the long QT syndrome: genes causing fainting and sudden death / G. M. Vincent // Annu. Rev. Med. - 1998. - Vol. 49. - P. 263-274.Vincent, G. M. The molecular genetics of the long QT syndrome: genes causing fainting and sudden death / G. M. Vincent // Annu. Rev. Med. - 1998. - Vol. 49. - P. 263-274.The long QT syndrome: a prospective international study / A. J. Moss [et al.] // Circulation. - 1985. - Vol. 71, № 1. - P. 17-21.The long QT syndrome: a prospective international study / A. J. Moss [et al.] // Circulation. - 1985. - Vol. 71, № 1. - P. 17-21.Khan, I. A. Long QT syndrome: Diagnosis and management / I. A. Khan // Ibid. - 2002. - Vol. l43, № 1. - P. 7-14.Khan, I. A. Long QT syndrome: Diagnosis and management / I. A. Khan // Ibid. - 2002. - Vol. l43, № 1. - P. 7-14.Evidence for a cardiac ion channel mutation underlying drug-induced QT prolongation and life-threatening arrhythmias / C. Napolilano [et al.] // J. Cardiovasc. Electrophysiol. - 2000. - Vol. 11. - P. 691-696.Evidence for a cardiac ion channel mutation underlying drug-induced QT prolongation and life-threatening arrhythmias / C. Napolilano [et al.] // J. Cardiovasc. Electrophysiol. - 2000. - Vol. 11. - P. 691-696.Non-invasive testing of acquired long QT syndrome: Evidence for multiple arrhytlimogenic substrates / P. Chevalier [et al.] // Cardiovasc. Res. - 2001.Non-invasive testing of acquired long QT syndrome: Evidence for multiple arrhytlimogenic substrates / P. Chevalier [et al.] // Cardiovasc. Res. - 2001.Бокерия, Л. А. Синдром удлиненного интервала QT - клиника, диагностика и лечение / Л. А. Бокерия, А. Ш. Ревишвили, И. В. Проничева // Анналы аритмологии. - 2005. - № 4. - С. 7-17.Бокерия, Л. А. Синдром удлиненного интервала QT - клиника, диагностика и лечение / Л. А. Бокерия, А. Ш. Ревишвили, И. В. Проничева // Анналы аритмологии. - 2005. - № 4. - С. 7-17.Макаров, Л. М. ЭКГ в педиатрии / Л. М. Макаров. - М.: Медпрактика, 2002. - 274 с.Макаров, Л. М. ЭКГ в педиатрии / Л. М. Макаров. - М.: Медпрактика, 2002. - 274 с.Schwartz, P. Idiopathic long QT syndrome: progress and guestions / P. Schwartz // Am Heart J. - 1985. - Vol. 109. - P. 399-411.Schwartz, P. Idiopathic long QT syndrome: progress and guestions / P. Schwartz // Am Heart J. - 1985. - Vol. 109. - P. 399-411.Макаров, Л. М. Холтеровское мониторирование / Л. М. Макаров. - М.: Медпрактика, 2000. - 216 с.Макаров, Л. М. Холтеровское мониторирование / Л. М. Макаров. - М.: Медпрактика, 2000. - 216 с.Школьникова, М. А. Синдром удлиненного интервала QT / М. А. Школьникова. - М.: Медпрактика, 2001.Школьникова, М. А. Синдром удлиненного интервала QT / М. А. Школьникова. - М.: Медпрактика, 2001.Risk stratification in the Long-QT Syndrome / S. G. Priori [et al.] // N. Engl. J. Med. - 2003. - Vol. 348, № 19. - P. 1866-1874.Risk stratification in the Long-QT Syndrome / S. G. Priori [et al.] // N. Engl. J. Med. - 2003. - Vol. 348, № 19. - P. 1866-1874.Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome / A. J. Moss [et al.] // Circulation. - 2000. - Vol. 15, № 101(6). - P. 616-623.Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome / A. J. Moss [et al.] // Circulation. - 2000. - Vol. 15, № 101(6). - P. 616-623.Association of long QT syndrome loci and cardiac events among the patients treated with в-blockers / S. G. Priory [et al.] // JAMA. - 2004. - Vol. 292 (15), № 11. - P. 1341-1344.Association of long QT syndrome loci and cardiac events among the patients treated with в-blockers / S. G. Priory [et al.] // JAMA. - 2004. - Vol. 292 (15), № 11. - P. 1341-1344.

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