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The QRS and T wave alternans — a new ECG pattern in Long QT syndrome
https://doi.org/10.33667/2078-5631-2024-30-10-14
Abstract
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.
About the Authors
L. M. Makarov
Federal 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 Agency
Russian Federation
Russian Federation
Leonid Makarov, MD, PhD Center for Syncope and Cardiac Arrthymias of Federal Scientific Clinical Center
A. G. Akopyan
Federal Scientific and Clinical Center for Children and Adolescents of the Federal Medico Biological Agency
Russian Federation
Russian Federation
Anush Akopyan, MD, Center for Syncope and Cardiac Arrthymias
E. V. Zaklyazminskaya
State 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. Bochkov
Russian Federation
Russian Federation
Elena V. Zaklyazminskaya, M.D., Ph.D., Head of Medical Genetics Laboratory
V. N. Komoliatova
Federal 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
Russian Federation
Russian Federation
Vera Komoliatova, MD, PhD, Center for Syncope and Cardiac Arrthymias of Federal
D. A. Besportochny
Federal Scientific and Clinical Center for Children and Adolescents of the Federal Medico Biological Agency
Russian Federation
Russian Federation
Dmitryi Besportochnyi, MD, Center for Syncope and Cardiac Arrthymias
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Review
For citations:
Makarov L.M., Akopyan A.G., Zaklyazminskaya E.V., Komoliatova V.N., Besportochny D.A. The QRS and T wave alternans — a new ECG pattern in Long QT syndrome. Medical alphabet. 2024;(30):10-14. (In Russ.) https://doi.org/10.33667/2078-5631-2024-30-10-14
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