medalphabetМедицинский алфавитMedical alphabet2078-56312949-2807ООО «Альфмед»10.33667/2078-5631-2024-30-10-14medalphabet-3985Research ArticleЭЛЕКТРОКАРДИОЛОГИЯЭЛЕКТРОКАРДИОЛОГИЯАльтернация комплекса QRS и зубца Т – новый электрокардиографический паттерн при синдроме удлиненного интервала QTThe QRS and T wave alternans — a new ECG pattern in Long QT syndromehttps://orcid.org/0000-0002-0111-3643МакаровЛ. М.MakarovL. M.

Макаров Леонид Михайлович, д.м.н., профессор, руководитель Центра синкопальных состояний и сердечных аритмий; профессор кафедры педиатрии

Leonid Makarov,  MD, PhD Center for Syncope and Cardiac Arrthymias of Federal Scientific Clinical Center

dr.leonidmakarov@mail.ruhttps://orcid.org/0000-0002-4867-0594АкопянА. Г.AkopyanA. G.

Врач Центра синкопальных состояний и сердечных аритмий 

Anush Akopyan, MD, Center for Syncope and Cardiac Arrthymias 

anushik.a.g@mail.ruЗаклязьминскаяЕ. В.ZaklyazminskayaE. V.

Д.м.н., заведующая лабораторией медицинской генетики; профессор кафедры медицинской генетики

Elena V. Zaklyazminskaya, M.D., Ph.D., Head of Medical Genetics Laboratory 

zhelene@mail.ruhttps://orcid.org/0000-0002-3691-7449КомолятоваВ. Н.KomoliatovaV. N.

Д.м.н., врач Центра синкопальных состояний и сердечных аритмий; профессор кафедры педиатрии

Vera Komoliatova, MD, PhD, Center for Syncope and Cardiac Arrthymias of Federal 

verakom@list.ruhttps://orcid.org/0000-0002-3699-2289БеспорточныйД. А.BesportochnyD. A.

Врач Центра синкопальных состояний и сердечных аритмий 

Dmitryi Besportochnyi, MD, Center for Syncope and Cardiac Arrthymias 

dr.blad@mail.ruФГБУ Федеральный научно-клинический центр детей и подростков ФМБА России ; ФГБОУ ДПО РМАНПО МЗ РФ Минздрава России ; ФГБУ ФНКЦ Академия постдипломного образования ФМБА РоссииРоссияFederal Scientific and Clinical Center for Children and Adolescents of the Federal Medico Biological Agency ; Federal State Budgetary Educational Institution of Higher Professional Education, Russian Medical Academy of Postgraduate Education of the Ministry of Health of the Russian Federation ; Federal State Budgetary Institution, Federal Scientific and Clinical Center, Academy of Postgraduate Education of the Federal Medico Biological AgencyRussian FederationФГБУ Федеральный научно-клинический центр детей и подростков ФМБА РоссииРоссияFederal Scientific and Clinical Center for Children and Adolescents of the Federal Medico Biological AgencyRussian FederationГНЦ ФГБНУ «РНЦХ им. акад. Б.В. Петровского» ; ФГБНУ «МГНЦ им. акад. Н.П. Бочкова»РоссияState Scientific Center, Federal State Budgetary Scientific Institution, Russian Scientific Center of Surgery named after Academician B.V. Petrovsky ; Federal State Budgetary Scientific Institution, Medical and Biological Center named after Academician N.P. BochkovRussian FederationФГБУ Федеральный научно-клинический центр детей и подростков ФМБА России ; ФГБОУ ДПО РМАНПО МЗ РФ Минздрава РоссииРоссияFederal Scientific and Clinical Center for Children and Adolescents of the Federal Medico Biological Agency ; Federal State Budgetary Educational Institution of Higher Professional Education, Russian Medical Academy of Postgraduate Education of the Ministry of Health of the Russian FederationRussian Federation202403122024030"Современная функциональная диагностика" (4)1014Copyright © Макаров Л.М., Акопян А.Г., Заклязьминская Е.В., Комолятова В.Н., Беспорточный Д.А., 20242024Макаров Л.М., Акопян А.Г., Заклязьминская Е.В., Комолятова В.Н., Беспорточный Д.А.Makarov L.M., Akopyan A.G., Zaklyazminskaya E.V., Komoliatova V.N., Besportochny D.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.med-alphabet.com/jour/article/view/3985

Представлен случай нового ЭКГ паттерна, у больной 6 лет с синдромом удлиненного интервала QT (СУИQT). На ЭКГ покоя удлинение корригированного интервала QT (QTc) до 568 мс. Проведенное молекулярно-генетическое исследование выявило мутацию de novo в гене SCN5A (p.Val1411Met), патогенный класс V, характерную для третьего молекулярно-генетического варианта СУИQT (LQT3). При холтеровском мониторировании отмечена «гиперадаптация» интервала QT (slope QT/RR 0.37 при норме до 0,24). Во время ночного сна, в периоды, соответствующие «быстрому сну» при классической полисомнографии, зарегистрированы эпизоды сочетанной альтернации зубца Т и альтернация комплекса QRS (АТ/АQRS). Данный паттерн ранее не описывался у больных с СУИQT. Назначен Атенолол 1 мг/кг. На фоне терапии через 2 месяца феномен АТ/АQRS не регистрировался. Обсуждаются возможные механизмы и прогностическое значение выявленного ЭКГ паттерна, вопросы лечения больных с LQT3. Заключение: Сочетанная альтернация зубца Т и комплекса QRS (АТ/АQRS) является новым, ранее не описанным, ЭКГ феноменом у больных с СУИQT, высоковероятно повышающим риск развития сердечных событий. «Гиперадаптация» интервала QT может быть одним из электрокардиологических признаков LQT3. Возникновение паттерна АТ/АQRS в фазу «быстрого сна», делает этот период, возможно, наиболее угрожаемым по развитию сердечных событий у больных с LQT3.

We present a case of a new ECG phenomenon that has not been described previously in patients with long QT syndrome (LQTS). Prolongation of the QT interval (QTc) to 568 ms were revealed in girl 6 years old on the resting ECG. There were no symptoms, syncope or family history of sudden death. A molecular genetic study revealed mutation «de novo» in the SCN5A gene (p.Val1411Met), a pathogenic class V that typical for the third molecular genetic variant of LQTS (LQT3). During Holter monitoring a «hyperadaptation» of the QT interval was noted (slope QT/RR 0.37 with normal limit till 0,24). New ECG pattern of the combination T wave alternans and QRS alternans (AT/AQRS) at the night sleep (possibly in REM sleep) were recorded. This pattern has not been described previously in patients with LQTS. Atenolol 1 mg/kg was prescribed. During therapy, AT/AQRS did not record after 2 months. Possible mechanisms and prognostic significance of the identified ECG pattern, issues of treatment of patients with LQT3 are discussed. Conclusions: Combined of T wave alternans and QRS alternans (AT/AQRS) is a new ECG pattern in patients with LQTS that is likely to increase the risk of cardiac events. “Hyperadaptation” of the QT interval may be one of the electrocardiological features of LQT3. The emergence of the AT/AQRS pattern during REM sleep makes this period perhaps the most dangerous for the development of cardiac events in patients with LQT3.

синдром удлиненного интервала QTLQT 3альтернация зубца Т и комплекса QRS«гиперадаптаия» интервала QTпериод снаlong QT syndromeLQT 3alternation of the T wave and QRS complexQT interval hyperadaptationsleep periodReferencesSchwartz P., Moss A., G.Vincent и R.Cramonю Diagnostic criteria for the long QT syndrome. An update1993. 88(2):782–4. DOI: 10.1161/01.cir.88.2.782.Schwartz P., Moss A., G.Vincent и R.Cramonю Diagnostic criteria for the long QT syndrome. An update1993. 88(2):782–4. DOI: 10.1161/01.cir.88.2.782.Crotti L, Celano G, Dagradi F, Schwartz P. Congenital long QT syndrome. Orphanet J Rare Dis 2008 Jul 7:3:18. doi: 10.1186/1750-1172-3-18.Crotti L, Celano G, Dagradi F, Schwartz P. Congenital long QT syndrome. Orphanet J Rare Dis 2008 Jul 7:3:18. doi: 10.1186/1750-1172-3-18.Moss A. The Long QT interval syndrome. The American Journal of Cardiology 1997;20:p.17–19. DOI: 10.1016/s0002-9149(97)00116-1.Moss A. The Long QT interval syndrome. The American Journal of Cardiology 1997;20:p.17–19. DOI: 10.1016/s0002-9149(97)00116-1.Макаров Л.М., Киселева И.И., Комолятова В.Н., Федина Н.Н.. Новые нормы и интерпретации детской электрокардиограммы. Педиатрия им. Г.Н. Сперанского. 2015; 94 (2).Makarov L.M., Kiseleva I.I., Komolyatova V.N., Fedina N.N.. New standards and interpretations of children electrocardiogram. Pediatria n.a. G.N. Speransky. 2015; 94 (2).Макаров Л.М., Комолятова В.Н., Мирошникова Е.Н., Казанцева М.А. Физиологическое значение и нормативные параметры частотной адаптации QT интервала при холтеровском мониторировании у здоровых молодых лиц. Кардиология 2008;48(4):54–8. PMID: 18447842.Makarov LM, Komoliatova VN, Miroshnikova EN, Kazantseva MA. Physiological significance and normative parameters of rate adaptation of QT-interval during holter monitoring in healthy persons of young age. Kardiologiia. 2008;48(4):54-8. Russian. PMID: 18447842.Макаров Л.М., Школьникова М.А., Березницкая В.В., Гиоргобиани Р.З. Структура ритма сердца у здоровых подростков по данным холтеровского мониторирования. Тер. Архив 1999;71(1):19–22. PMID: 10097294.Makarov LM, Shkol’nikova MA, Bereznitskaia VV, Giorgobiani RR. Struktura ritma serdtsa v period sna u zdorovykh podrostkov po dannym kholterovskogo monitorirovaniia EKG [Cardiac rhythm in sleeping healthy adolescents as recorded by holter ECG monitoring]. Ter Arkh. 1999;71(1):19-22. PMID: 10097294.Priori SG, Blomström-Lundqvist C, Mazzanti A, Blom N, Borggrefe M, Camm J, et al. ESC Scientific Document Group. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Pediatric and Congenital Cardiology (AEPC). Eur Heart J. 2015 Nov 1;36(41):2793–2867. doi: 10.1093/eurheartj/ehv316.Priori SG, Blomström-Lundqvist C, Mazzanti A, Blom N, Borggrefe M, Camm J, et al. ESC Scientific Document Group. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death: The Task Force for the Management of Patients with Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death of the European Society of Cardiology (ESC). Endorsed by: Association for European Pediatric and Congenital Cardiology (AEPC). Eur Heart J. 2015 Nov 1;36(41):2793–2867. doi: 10.1093/eurheartj/ehv316.Bennett PB, Yazawa K, Makita N, George AL Jr. Molecular mechanism for an inherited cardiac arrhythmia. Nature. 1995 Aug 24;376(6542):683–5. doi: 10.1038/376683a0.Bennett PB, Yazawa K, Makita N, George AL Jr. Molecular mechanism for an inherited cardiac arrhythmia. Nature. 1995 Aug 24;376(6542):683–5. doi: 10.1038/376683a0.Horne AJ, Eldstrom J, Sanatani S, Fedida D. A novel mechanism for LQT3 with 2:1 block: a pore-lining mutation in Nav1.5 significantly affects voltage-dependence of activation. Heart Rhythm. 2011;8(5):770–777. doi:10.1016/j.hrthm.2010.12.041.Horne AJ, Eldstrom J, Sanatani S, Fedida D. A novel mechanism for LQT3 with 2:1 block: a pore-lining mutation in Nav1.5 significantly affects voltage-dependence of activation. Heart Rhythm. 2011;8(5):770–777. doi:10.1016/j.hrthm.2010.12.041.Moss AJ, Zareba W, Benhorin J, Locati EH, Hall WJ, Robinson JL, Schwartz PJ, Towbin JA, Vincent GM, Lehmann MH. ECG T-wave patterns in genetically distinct forms of the hereditary long QT syndrome. Circulation. 1995 Nov 15;92(10):2929–34. doi: 10.1161/01.cir.92.10.2929.Moss AJ, Zareba W, Benhorin J, Locati EH, Hall WJ, Robinson JL, Schwartz PJ, Towbin JA, Vincent GM, Lehmann MH. ECG T-wave patterns in genetically distinct forms of the hereditary long QT syndrome. Circulation. 1995 Nov 15;92(10):2929–34. doi: 10.1161/01.cir.92.10.2929.Stramba-Badiale M, Locati EH, Martinelli A, Courville J, Schwartz PJ. Gender and the relationship between ventricular repolarization and cardiac cycle length during 24-h Holter recordings. Eur Heart J. 1997 Jun;18(6):1000-6. doi: 10.1093/oxfordjournals.eurheartj.a015357.Stramba-Badiale M, Locati EH, Martinelli A, Courville J, Schwartz PJ. Gender and the relationship between ventricular repolarization and cardiac cycle length during 24-h Holter recordings. Eur Heart J. 1997 Jun;18(6):1000-6. doi: 10.1093/oxfordjournals.eurheartj.a015357.Makarov L, Komoliatova V, Zevald S, Schmidt G, Muller CA, Serebruany V. QT dynamicity, microvolt T-wave alternans, and heart rate variability during 24- hour ambulatory electrocardiogram monitoring in the healthy newborn of first to fourth day of life. J Electrocardiol. 2010 Jan-Feb;43(1):8-14. doi: 10.1016/j.jelectrocard.2009.11.001.Makarov L, Komoliatova V, Zevald S, Schmidt G, Muller CA, Serebruany V. QT dynamicity, microvolt T-wave alternans, and heart rate variability during 24- hour ambulatory electrocardiogram monitoring in the healthy newborn of first to fourth day of life. J Electrocardiol. 2010 Jan-Feb;43(1):8-14. doi: 10.1016/j.jelectrocard.2009.11.001.Malik M. ECG and VT/VF symposium. J Electrocardiol. 2010 Jan-Feb;43(1):1-3. doi: 10.1016/j.jelectrocard.2009.11.003.Malik M. ECG and VT/VF symposium. J Electrocardiol. 2010 Jan-Feb;43(1):1-3. doi: 10.1016/j.jelectrocard.2009.11.003.Schwartz PJ, Priori SG, Spazzolini C, Moss AJ, Vincent GM, Napolitano C, et al. Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation. 2001 Jan 2;103(1):89-95. doi: 10.1161/01.cir.103.1.89. PMID: 11136691.Schwartz PJ, Priori SG, Spazzolini C, Moss AJ, Vincent GM, Napolitano C, et al. Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias. Circulation. 2001 Jan 2;103(1):89-95. doi: 10.1161/01.cir.103.1.89. PMID: 11136691.Hering H.E. Das Wesen des Herzalternans. Munchen Med Wochenshr, 4, (1908)Hering H.E. Das Wesen des Herzalternans. Munchen Med Wochenshr, 4, (1908)Lewis Т. Notes upon alternation of the heart. Q J Med, 4, pp. 141–144 (1910).Lewis Т. Notes upon alternation of the heart. Q J Med, 4, pp. 141–144 (1910).Kalter H.H., Schwartz M.L. Electrical alternans N Y State J Med, (1948).Kalter H.H., Schwartz M.L. Electrical alternans N Y State J Med, (1948).Surawicz B, Fisch C. Cardiac alternans: diverse mechanisms and clinical manifestations. J Am Coll Cardiol. 1992 Aug;20(2):483-99. doi: 10.1016/0735-1097(92)90122-4.Surawicz B, Fisch C. Cardiac alternans: diverse mechanisms and clinical manifestations. J Am Coll Cardiol. 1992 Aug;20(2):483-99. doi: 10.1016/0735-1097(92)90122-4.Pernot C, Henry M, Debruille C. Les syndromes cardio-auditifs d’origine génétique [Cardio-auditory syndromes of genetic origin]. Coeur Med Interne. 1974 JulSep;13(3):429-43. French. PMID: 4140046.Pernot C, Henry M, Debruille C. Les syndromes cardio-auditifs d’origine génétique [Cardio-auditory syndromes of genetic origin]. Coeur Med Interne. 1974 JulSep;13(3):429-43. French. PMID: 4140046.Zareba W, Moss AJ, le Cessie S, Hall WJ. T wave alternans in idiopathic long QT syndrome. J Am Coll Cardiol. 1994 Jun;23(7):1541-6. doi: 10.1016/0735-1097(94)90653-x. PMID: 8195511.Zareba W, Moss AJ, le Cessie S, Hall WJ. T wave alternans in idiopathic long QT syndrome. J Am Coll Cardiol. 1994 Jun;23(7):1541-6. doi: 10.1016/0735-1097(94)90653-x. PMID: 8195511.Makarov L, Komoliatova V, Zaklyazminskaya E, Dmitrieva A. Ambulatory ECG Monitoring in Patients With Long QT Syndrome. Conference: Scientific Session AHA. Su 3177. Chicago 10-12 November 2018. Su3177 | Sunday, November 11, 2018. DOI: 10.13140/RG.2.2.32275.69921.Makarov L, Komoliatova V, Zaklyazminskaya E, Dmitrieva A. Ambulatory ECG Monitoring in Patients With Long QT Syndrome. Conference: Scientific Session AHA. Su 3177. Chicago 10-12 November 2018. Su3177 | Sunday, November 11, 2018. DOI: 10.13140/RG.2.2.32275.69921.Hamburger W, Katz L, Sahir O. Electrical alternans. A clinical study with a report of two necropsies. Jour A.M.A. 1936, CVI, 902.Hamburger W, Katz L, Sahir O. Electrical alternans. A clinical study with a report of two necropsies. Jour A.M.A. 1936, CVI, 902.White P. «Heart disease» (Sec ed.) NY, The Macmillan Company. 1938; p.128-129.White P. «Heart disease» (Sec ed.) NY, The Macmillan Company. 1938; p.128-129.Сигал А.М. Ритмы сердечной деятельности и их нарушения. 1935, Одесса. 367 с.Seagal A.M. Rhythms of cardiac activity and their disorders. 1935, Odessa. 367 pp.Spodick DH. Electric alternation of the heart. Its relation to the kinetics and physiology of the heart during cardiac tamponade. Am J Cardiol. 1962 Aug;10:155-65. doi: 10.1016/0002-9149(62)90290-4.Spodick DH. Electric alternation of the heart. Its relation to the kinetics and physiology of the heart during cardiac tamponade. Am J Cardiol. 1962 Aug;10:155-65. doi: 10.1016/0002-9149(62)90290-4.Kleinfeld M, Stein E, Kossmann CE.. Electrical alternans with emphasis on recent observations made by means of single-cell electrical recording. Am Heart J. 1963 Apr;65:495-500. doi: 10.1016/0002-8703(63)90099-1.Kleinfeld M, Stein E, Kossmann CE.. Electrical alternans with emphasis on recent observations made by means of single-cell electrical recording. Am Heart J. 1963 Apr;65:495-500. doi: 10.1016/0002-8703(63)90099-1.Brembilla-Perrot B, Lucron H, Schwalm F, Haouzi A. Mechanism of QRS electrical alternans. Heart. 1997 Feb;77(2):180-2. doi: 10.1136/hrt.77.2.180.Brembilla-Perrot B, Lucron H, Schwalm F, Haouzi A. Mechanism of QRS electrical alternans. Heart. 1997 Feb;77(2):180-2. doi: 10.1136/hrt.77.2.180.Green M, Heddle B, Dassen W, Wehr M, Abdollah H, Brugada P, Wellens HJ. Value of QRS alteration in determining the site of origin of narrow QRS supraventricular tachycardia. Circulation. 1983 Aug;68(2):368-73. doi: 10.1161/01.cir.68.2.368. PMID: 6861312.Green M, Heddle B, Dassen W, Wehr M, Abdollah H, Brugada P, Wellens HJ. Value of QRS alteration in determining the site of origin of narrow QRS supraventricular tachycardia. Circulation. 1983 Aug;68(2):368-73. doi: 10.1161/01.cir.68.2.368. PMID: 6861312.Kremers MS, Miller JM, Josephson ME. Electrical alternans in wide complex tachycardias. Am J Cardiol. 1985 Aug 1;56(4):305-8. doi: 10.1016/0002-9149(85)90854-9.Kremers MS, Miller JM, Josephson ME. Electrical alternans in wide complex tachycardias. Am J Cardiol. 1985 Aug 1;56(4):305-8. doi: 10.1016/0002-9149(85)90854-9.Chinushi M, Hosaka Y, Washizuka T, Furushima H, Aizawa Y. Arrhythmogenesis of T wave alternans associated with surface QRS complex alternans and the role of ventricular prematurity: observations from a canine model of LQT3 syndrome. J Cardiovasc Electrophysiol. 2002 Jun;13(6):599-604. doi: 10.1046/j.1540-8167.2002.00599.x. PMID: 12108505.Chinushi M, Hosaka Y, Washizuka T, Furushima H, Aizawa Y. Arrhythmogenesis of T wave alternans associated with surface QRS complex alternans and the role of ventricular prematurity: observations from a canine model of LQT3 syndrome. J Cardiovasc Electrophysiol. 2002 Jun;13(6):599-604. doi: 10.1046/j.1540-8167.2002.00599.x. PMID: 12108505.Caples SM, Rosen CL, Shen WK, Gami AS, Cotts W, Adams M, Dorostkar P, Shivkumar K, Somers VK, Morgenthaler TI, Stepanski EJ, Iber C. The scoring of cardiac events during sleep. J Clin Sleep Med. 2007 Mar 15;3(2):147–54.Caples SM, Rosen CL, Shen WK, Gami AS, Cotts W, Adams M, Dorostkar P, Shivkumar K, Somers VK, Morgenthaler TI, Stepanski EJ, Iber C. The scoring of cardiac events during sleep. J Clin Sleep Med. 2007 Mar 15;3(2):147–54.

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