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”10.21508/1027-4065-2024-69-3-6-18perinatology-1997Research ArticleПЕРЕДОВАЯEDITORIALВнезапная сердечная смерть при гипертрофической кардиомиопатии у детей: подходы к профилактикеSudden cardiac death in children with hypertrophic cardiomyopathy: approaches to preventionhttps://orcid.org/0000-0002-5273-6859ЛеонтьеваИ. В.LeontyevaI. V.

Леонтьева Ирина Викторовна — д.м.н., проф.,гл. науч. сотр. отдела детской кардиологии и аритмологии; проф. кафедры инновационной педиатрии и детской хирургии

125412 Москва, ул. Талдомская, д. 2

ОСП «Научно-исследовательский клинический институт педиатрии и детской хирургии им. академика Ю.Е. Вельтищева» (Институт Вельтищева) ФГАОУ ВО РНИМУ им. Н.И. Пирогова Минздрава России; ФГАОУ ВО «Российский национальный исследовательский медицинский университет им. Н.И. Пирогова» Минздрава РоссииРоссияVeltischev Research and Clinical Institute for Pediatrics and Pediatric Surgery of the Pirogov Russian National Research Medical University; Pirogov Russian National Research Medical UniversityRussian Federation202411072024693618Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20242024Ltd. “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/1997

Гипертрофическая кардиомиопатия — основная причина внезапной сердечной смерти у детей и подростков, которая в большинстве случаев обусловлена жизнеугрожающими аритмиями. В статье описаны факторы риска внезапной смерти и освещены основные проблемы профилактики внезапной сердечной смерти у детей с гипертрофической кардиомиопатией. Рассмотрены современные проблемы стратификации риска внезапной сердечной смерти у детей, основанные на оценке факторов риска и многофакторных математических моделях риска. Рассмотрены клинические, функциональные и генетические маркеры риска внезапной смерти у детей в сравнении со взрослой популяцией. Представлены показания к имплантации кардиовертера-дефибриллятора (ИКД) для профилактики внезапной смерти и оценена эффективность ИКД-терапии.

Hypertrophic cardiomyopathy is the leading cause of sudden cardiac death in children and adolescents, which in most cases is caused by life-threatening arrhythmias. The article highlights the main risk factors and problems of preventing sudden cardiac death in children with hypertrophic cardiomyopathy. The modern problems of stratification of the risk of sudden cardiac death in children based on the assessment of risk factors and multifactorial mathematical models of risk are considered. The clinical, functional and genetic markers of the risk of sudden death in children in comparison with the adult population are considered. Indications for cardioverter defibrillator implantation for the prevention of sudden death are presented and its effectiveness is evaluated.

детигипертрофическая кардиомиопатиявнезапная смертьфакторы риска внезапной сердечной смертикардиовертер-дефибрилляторChildrenhypertrophic cardiomyopathysudden deathrisk factors for sudden cardiac deathcardioverter-defibrillatorReferencesElliott P.M., Anastasakis A., Borger M.A., Borggrefe M., Cecchi F., Charron P. еt al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J 2014; 35(39): 2733–2779. DOI: 10.1093/eurheartj/ehu284Elliott P.M., Anastasakis A., Borger M.A., Borggrefe M., Cecchi F., Charron P. еt al. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC). Eur Heart J 2014; 35(39): 2733–2779. 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The authors declare that there are no conflicts of interest present.