perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”10.21508/1027-4065-2014-59-6-35-49perinatology-279Research ArticleКАРДИОЛОГИЯCARDIOLOGYСинкопальные состояния у детей с гипертрофической кардиомиопатиейSyncope in children with hypertrophic cardiomyopathyЛеонтьеваИ. В.LeontyevaI. V.

д.м.н., проф., рук. отделения патологии сердечно-сосудистой системы НИКИ педиатрии

МакароваВ. А.MakarovaV. A.

асп. того же отдела

ТермосесовС. А.TermosesovS. A.

зав. отделением хирургического лечения сложных нарушений сердечного ритма указанного учреждения 125412 Москва, ул. Талдомская, д. 2

Научно-исследовательский клинический институт педиатрии, МоскваРоссияResearch Clinical Institute of Pediatrics, MoscowRussian Federation2014170320165965765Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20162016Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/279

Обследованы 70 детей в возрасте от 7 до 17 лет с гипертрофической кардиомиопатией; среди них у 11 выявлены синкопальные состояния и у 5 — пресинкопальные. Программа обследования включала: стандартную ЭКГ, допплерэхокардиографию, холтеровское мониторирование суточной ЭКГ, пробу с дозированной физической нагрузкой (тредмил-тест по протоколу Bruse). Определялись маркеры электрической нестабильности миокарда. Установлено, что синкопальные состояния у детей с гипертрофической кардиомиопатией гетерогенны, в большинстве случаев имеют аритмогенное происховдение, возникают на фоне тахи- (44%) или брадиаритмий (25%), у '/3 обследуемых вероятен вазовагальный генез. Для детей с синкопальными состояниями характерна асимметричная, обструктивная форма кардиомиопатии, при этом часто имеет место комбинация гипертрофии левого желудочка с расширением полости левого предсердия. По данным холтеровского мониторирования, у пациентов преобладала брадикардия, у 3 детей выявлены паузы сердечного ритма более 2 с, обусловленные атриовент-рикулярной блокадой 2-й степени (у 1), синдромом слабости синусового узла (у 2). Тахизависимые нарушения сердечного ритма отмечены у двух пациентов. Для детей с синкопальными состояниями характерны признаки электрической нестабильности миокарда в виде редукции ранней фазы турбулентности ритма сердца, нарушение адаптации интервала QT/RR по типу «гиперадаптации». Представлена разработанная тактика ведения детей с синкопальными состояниями, показания для имплантации кардиовертердефибриллятора, электрокардиостимулятора, петлевого регистратора электрокардиограммы.

Seventy children aged 7 to 17 years with hypertrophic cardiomyopathy (HCM) were examined; among them there were 11 syncope patients and 5 presyncope patients. The screening program included standard electrocardiography (ECG), Doppler echocardiogra-phy, 24-hour Holter ECG monitoring, and an incremental exercise testing (Bruce treadmill test). The markers of myocardial electrical instability were determined. In the children with HCM, syncope was established to be heterogeneous; it had an arrhythmogenic origin and, in most cases, occurred in the presence of tachyarrhythmia (44%) or bradyarrythmia (25%); its vasovagal genesis was probable in one third of the examinees. The children with syncope were typified by the asymmetric, obstructive form of HCM, at the same tone there was most commonly left ventricular hypertrophy concurrent with left atrial enlargement. 24-hour Holter monitoring showed that bradycardia was prevalent in the patients, 3 patients were found to have more than 2-second cardiac rhythm pauses caused by second-degree atrioventricular block in 1 case or by sick sinus syndrome in 2. Nonsustained ventricular tachycardia was noted in two patients. The children with syncope were typified by the signs of myocardial electrical instability as a reduction in the early phase of heart rate turbulence and by impaired QT/RR interval adaptation as hyperadaptation. The paper presents the developed management tactics for children with syncope and indications for the implantation of a cardioverter defibrillator, a pacemaker, or an ECG loop recorder.

детиподросткигипертрофическая кардиомиопатиясинкопальные состояниянарушения сердечного ритма и проводимостиэлектрическая нестабильность миокардапрофилактика внезапной сердечной смерти.childrenadolescentshypertrophic cardiomyopathysyncopecardiac arrhythmia and conduction disturbancesmyocardial electrical instabilityprevention of sudden cardiac death.ReferencesElliott P.M., Anastasakis A., Borger M.A. et al. 2014 ESC Guidelines on diagnosis and management of hypertropliic cardiomyopathy The Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology. Eur Heart J 2014; doi:10.1093/eurheartj/ehu284. www escardio.org/ guidelines.Elliott P.M., Anastasakis A., Borger M.A. et al. 2014 ESC Guidelines on diagnosis and management of hypertropliic cardiomyopathy The Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology. 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The authors declare that there are no conflicts of interest present.