cardioКардиологияKardiologiia0022-90402412-5660Kardiomag10.18087/cardio.2018.12.10191cardio-375Research ArticleАРИТМИИ СЕРДЦАCARDIAC ARRHYTHMIASИмплантация кардиовертеров-дефибрилляторов у детей с синдромом удлиненного интервала QT: оценка показаний, эффективности и безопасности на основе 10-летнего опытаImplantation of Cardioverter-Defibrillator in Children With Long-QT Syndrome: Assessment of Indications, Efficacy, and Safety Based on 10-Year ExperienceИльдароваР. А.IldarovaR. A.

Москва

Ildarova Rukizhat A. - PhD, MD.

Moscow

ildarova@pedklin.ruШкольниковаМ. А.ShkolnikovaM. A.

Москва

Moscow

ТермосесовС. А.TermosesovS. A.

Москва

Moscow

ОСП Научно­-исследовательский клинический институт педиатрии им. акад. Ю. Е. Вельтищева; ФГБОУ ВО «РНИМУ им. Н.И. Пирогова» Минздрава РоссииРоссияClinical Institute of Pediatrics named after Academician Y. E. Veltishev; RNIMU after N.I. PirogovRussian FederationОСП Научно-­исследовательский клинический институт педиатрии им. акад. Ю. Е. Вельтищева; ФГБОУ ВО «РНИМУ им. Н.И. Пирогова» Минздрава РоссииРоссияClinical Institute of Pediatrics named after Academician Y. E. Veltishev; RNIMU after N.I. PirogovRussian Federation20182512201858125258Copyright © Kardiomag, 20182018KardiomagKardiomaghttps://cardio.elpub.ru/jour/about/submissions#copyrightNoticehttps://cardio.elpub.ru/jour/article/view/375Цель исследования

Цель исследования. Оценка особенностей течения синдрома удлиненного интервала QT в детском возрасте до и после имплантации кардиовертера­дефибриллятора (ИКД) и оптимизация показания к ИКД­терапии.

Материалы и методы

Материалы и методы. В исследование были включены 48 детей с синдромом удлиненного интервала QT из 44 неродственных семей, которым был имплантирован кардиовертер­дефибриллятор (28 мальчиков и 20 девочек); средний возраст имплантации составил 11,8±3,8 года. Длительность катамнеза после имплантации ИКД составила в среднем 5,2±2,8 года. В группу сравнения вошли 59 детей с синдромом удлиненного интервала QT из 46 неродственных семей, сопоставимые по возрасту и полу с пациентами основной группы, получающие антиаритмическую терапию β­адреноблокаторами. Оценивали клинические и электрокардиографические характеристики заболевания при первичном визите и в динамике.

Результаты

Результаты. Больные с синдромом удлиненного интервала QT и ИКД в детском возрасте – это преимущественно пробанды, имеющие интервал QTc более 500 мс, рецидивирующие синкопальные состояния и нередко внезапную остановку кровообращения в анамнезе, нуждающиеся в высоких дозах β­адреноблокаторов для контроля желудочковых тахиаритмий. Вывод. Имплантация кардиовертера­дефибриллятора является эффективным и безопасным методом как первичной, так вторичной профилактики внезапной сердечной смерти у детей с синдромом удлиненного интервала QT.

Purpose

Purpose: to assess specificities of course of the long­QT syndrome in children before and after implantation of cardioverter­defibrillator (ICD), and optimization of indications to ICD­therapy.

Materials and methods

Materials and methods. We included in this study 48 children with long­QT syndrome from 44 unrelated families (28 boys and 20 girls), who underwent ICD implantation at the mean age 11.8±3.8 years. Mean duration of follow­up after implantation was 5.2±2.8 years. Data from these children were compared with those from 59 children of comparable age and gender with long­QT syndrome from 46 unrelated families receiving antiarrhythmic therapy (β­adrenoblockers). We assessed clinical and electrocardiographic characteristics of the disease obtained at initial visit and their dynamics thereafter.

Results

Results. Children with long­QT syndrome and ICD were mainly probands with interval QT longer than 500 ms, recurrent syncope and often history of sudden cardiac arrest requiring high doses of β­adrenoblockers for control of ventricular tachyarrhythmias.

Conclusion

Conclusion. ICD implantation is an effective and safe method both of primary and secondary prevention of sudden cardiac death in children with long­QT syndrome.

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