Syncope during sleep in a child with hereditary long QT syndrome (case report)

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Genetically determined long QT syndrome is characterized by attacks of loss of consciousness against the background of episodes of malignant ventricular arrhythmias. Due to the high risk of sudden cardiac death, timely diagnosis of this pathology is of great importance. In the given clinical observation, the manifestation of the syndrome of an extended QT interval in a 5-year-old boy is described. The manifestations of the disease were short-term episodes of loss of consciousness, which developed during sleep in the morning during the transition of sleep phases: "sleep - wakefulness". Clinically, this was expressed by crying, straining, pallor of the skin, followed by a complete loss of muscle tone and involuntary urination. Examination on a standard electrocardiogram revealed a significant lengthening of the absolute and corrected QT interval. During the daily monitoring of the electrocardiogram, pre-morning paroxysm of frequent ventricular extrasystole was revealed, against which a burst of tachycardia "pirouette" was recorded with a heart rate of 138 beats / min, lasting 8 seconds. The patient was prescribed antiarrhythmic therapy, against the background of which a positive effect was obtained in the form of the disappearance of clinical symptoms, a reduction in the duration of the QT interval (absolute and corrected), the absence of bursts of ventricular tachycardia "pirouette" with repeated daily recordings of the electrocardiogram. Clinical observation demonstrates the need for timely in-depth cardiological examination of patients with syncope.

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Irina I. Pshenichnikova

Russian Medical Academy of Continuous Professional Education

Moscow, Russia

Tatiana M. Tvorogova

Russian Medical Academy of Continuous Professional Education

Moscow, Russia

Irina N. Zakharova

Russian Medical Academy of Continuous Professional Education

Moscow, Russia


  1. Ильдарова Р.А., Школьникова М.А. Современная тактика ведения пациентов молодого возраста с синдромом удлиненного интервала QT: от ранней диагностики к имплантации кардиовертера-дефибриллятора и мониторингу маркеров риска внезапной смерти. Сибирский мед. журн. 2015; 30 (1).
  2. Kobza R et al. Prevalence of long and short QT in a young population of 41,767 predominantly male Swiss conscripts. Heart rhythm 2009; 6 (5): 652-7.
  3. Schwartz PJ. Idiopathic long QT syndrome: progress and questions. Am heart j 1985; 109 (2): 399-411.
  4. Школьникова М.А., Харлап М.С., Ильдарова Р.А. Генетически детерминированные нарушения ритма сердца. Рос. кардиологический журн. 2011; 1.
  5. Goldenberg I. Genotype-Phenotype Correlation in Congenital LQTS: Implications for Diagnosis and Risk Stratification. Cardiac Repolarization. Springer, Cham; 2020: p. 141-64.
  6. Макаров Л.М. Клинико-электрокардиографические синдромы с оличком внезапной сердечной смерти. Синдром удлиненного интервала QT. Мед. алфавит. 2016; 3 (30): 12-8.
  7. Schwartz PJ et al. Genotype-phenotype correlation in the long-QT syndrome: genespecific triggers for life-threatening arrhythmias. Circulation 2001; 103 (1): 89-95.
  8. Школьникова М.А., Миклашевич И.М., Калинин Л.А. Нормативные параметры ЭКГ у детей и подростков. М.: Ассоциация детских кардиологов России, 2010.
  9. Ильдарова РА., Школьникова М.А. Врожденный синдром удлиненного интервала QT как проявление первичной электрической патологии сердца. Рос. вестник перинатологии и педиатрии. 2010; 55 (2).
  10. Schwartz PJ et al. Long QT syndrome patients with mutations of the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate: implications for gene-specific therapy. Circulation 1995; 92 (12): 3381-6.
  11. Макаров Л.М. Холтеровское мониторирование. Т 409. 4-е изд. M.: МЕД-ПРАКТИКА-М, 2017.
  12. Moss AJ et al. Effectiveness and limitations of p-blocker therapy in congenital long-QT syndrome. Circulation 2000; 101 (6): 616-23.

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