cardiovascularКардиоваскулярная терапия и профилактикаCardiovascular Therapy and Prevention1728-88002619-0125«SILICEA-POLIGRAF» LLC10.15829/1728-8800-2018-2-101-124cardiovascular-805Research ArticleКЛИНИЧЕСКИЕ РЕКОМЕНДАЦИИCLINICAL GUIDELINESСОВРЕМЕННАЯ СТРАТЕГИЯ ТЕРАПИИ ЛЕГОЧНОЙ ГИПЕРТЕНЗИИ У ДЕТЕЙCONTEMPORARY STRATEGY OF PULMONARY HYPERTENSION MANAGEMENT IN PEDIATRICSМиклашевичИ. М.MiklashevichI. M.

Кандидат медицинских наук, заведующий детским кардиологическим отделением обособленного структурного подразделения.

Москва

Moscow

imiklashevich@pedklin.ruШкольниковаМ. А.ShkolnikovaМ. A.

Доктор медицинских наук, профессор, научный руководитель обособленного структурного подразделения.

Москва

Moscow

ГорбачевскийС. В.GorbachevskyS. V.

Доктор медицинских наук, профессор, директор экспертного Центра хирургического и эндоваскулярного лечения легочной гипертензии, заведующий отделением хирургического лечения заболеваний сердца с прогрессирующей легочной гипертензией.

Москва

Moscow

ШмальцА. А.ShmaltsA. A.

Доктор медицинских наук, ведущий научный сотрудник отделения хирургического лечения заболеваний сердца с прогрессирующей легочной гипертензией.

Москва

Moscow

ГрозноваО. С.GroznovaО. S.

Доктор медицинских наук, ведущий научный сотрудник отдела детской кардиологии и аритмологии обособленного структурного подразделения.

Москва

Moscow

СадыковаД. И.SadykovaD. I.

Доктор медицинских наук, профессор, заведующая кафедрой педиатрии с курсом поликлинической педиатрии, генеральный директор ВОО “Ассоциация детских кардиологов России”, заслуженный врач РФ.

Казань

ЯковлеваЛ. В.YakovlevaL. V.

Доктор медицинских наук, профессор, заведующая кафедрой поликлинической и неотложной педиатрии с курсом института дополнительного профессионального образования.

Уфа

 

Ufa

ДегтяревД. Н.DegtyarevD. N.

Доктор медицинских наук, профессор, заместитель директора по научной работе, заведующий кафедрой неонатологии.

Москва

Moscow

БуровА. А.BurovA. A.

Заведующий по клинической работе отделения хирургии новорожденных отдела неонатологии и педиатрии, врач анестезиолог­реаниматолог высшей категории.

Москва

Moscow

ОвсянниковД. Ю.OvsyannikovD. Yu.

Доктор медицинских наук, профессор, заведующий кафедрой педиатрии.

Москва

Moscow

ВолковА. В.VolkovA. V.

Кандидат медицинских наук, заведующий лабораторией инструментальной и УЗ­диагностики.

Москва

Moscow

Научно-исследовательский клинический институт педиатрии им. акад. Ю.Е. Вельтищева, ФГБОУ ВО РНИМУ им. Н. И. Пирогова Минздрава РоссииVeltishchev Scientific­Research Clinical Institute of Pediatrics of the Pirogov RNRMUНаучно-исследовательский клинический институт педиатрии им. акад. Ю.Е. Вельтищева,ФГБОУ ВО РНИМУ им. Н. И. Пирогова Минздрава РоссииVeltishchev Scientific­Research Clinical Institute of Pediatrics of the Pirogov RNRMUФГБУ “Национальный медицинский исследовательский центр сердечно-сосудистой хирургии им. А. Н. Бакулева” Минздрава РоссииBakulev Scientific Center of CardiosurgeryФГБУ “Национальный медицинский исследовательский центр сердечно-сосудистой хирургии им. А. Н. Бакулева” Минздрава РоссииBakulev Scientific Center of CardiosurgeryНаучно-исследовательский клинический институт педиатрии им. акад. Ю. Е. Вельтищева,ФГБОУ ВО РНИМУ им. Н. И. Пирогова Минздрава РоссииVeltishchev Scientific­Research Clinical Institute of Pediatrics of the Pirogov RNRMUГБОУ ДПО КГМА Минздрава РоссииKazanskaya State Medical AcademyБашкирский государственный медицинский университетBashkirskiy State Medical UniversityФГБУ “Национальный медицинский исследовательский центр акушерства, гинекологии и перинатологии им. В.И. Кулакова Минздрава РоссииKulakov Scientific Center of Obstetrics, Gynecology and PerinatologyФГАОУ ВО Российский университет дружбы народовRUDN UniversityФГБНУ НИИР им. В.А. НасоновойNasonova Scientific­Research Center of Rheumatology201820042018172101124Copyright © Миклашевич И.М., Школьникова М.А., Горбачевский С.В., Шмальц А.А., Грознова О.С., Садыкова Д.И., Яковлева Л.В., Дегтярев Д.Н., Буров А.А., Овсянников Д.Ю., Волков А.В., 20182018Миклашевич И.М., Школьникова М.А., Горбачевский С.В., Шмальц А.А., Грознова О.С., Садыкова Д.И., Яковлева Л.В., Дегтярев Д.Н., Буров А.А., Овсянников Д.Ю., Волков А.В.Miklashevich I.M., Shkolnikova М.A., Gorbachevsky S.V., Shmalts A.A., Groznova О.S., Sadykova D.I., Yakovleva L.V., Degtyarev D.N., Burov A.A., Ovsyannikov D.Y., Volkov A.V.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://cardiovascular.elpub.ru/jour/article/view/805

Легочная гипертензия (ЛГ) у детей — полигенное, многофакторное состояние с крайне неблагоприятным прогнозом. Выбор оптимальной тактики ведения является трудной задачей. В отсутствие лечения средняя выживаемость у детей не превышает одного года. В последние два десятилетия революционные изменения, произошедшие в лечебной тактике, улучшили выживаемость этой группы пациентов. В настоящее время педиатры и детские кардиологи имеют на вооружение три группы препаратов, воздействующие на основные пути патогенеза ЛГ: путь эндотелина, путь оксида азота и путь простациклина. В настоящее время терапия легочной артериальной гипертензии у детей в значительной степени основывается на результатах исследований, полученных у взрослых пациентов; однако не так давно были проведены первые рандомизированные исследования с участием детей. Коллектив авторов представляет современный взгляд на проблему ЛГ у детей и рекомендации экспертов по лечению детей с этой патологией. Класс рекомендаций и уровень доказательности определялся по общепринятой методике на основании данных, полученных при исследовании детской популяции, или на основании данных исследований с участием взрослых, в которые были включены более 10% детей. В основе разработанной российскими учеными­клиницистами стратегии лечения ЛГ в детском возрасте лежит анализ опыта ведения пациентов с этой патологией в России, существующих практик и клинических рекомендаций по лечению детей с ЛГ в Европе, а также данные опубликованных современных исследований в этой области.

Pulmonary hypertension (PH) in pediatrics is a polygenic multifactorial condition with extremely adverse prognosis. Selection of optimal management is a severe task. In absence of treatment the mean life duration in children is not higher one year. Last two decades, revolution in approaches to treatment improved the survival of this patients group. Recently, pediatricians and pediatric cardiologists have three drugs groups that act on the main pathogenetic chains of PH: endothelin pathway, nitric oxide pathway and prostacyclin pathway. At the moment, approaches to pediatric PH are based on the data obtained in the trials on adult patients. However, not long ago there were first randomized trials on children performed. The group of authors of current article presents a modern view on the problem of PH in children, and expert recommendations on children management. Class of recommendations and evidence level were set by the data obtained in pediatric population or on adult population with at least 10% of children included. To the strategy, developed by the Russian clinicians, laid the analysis of experience of the pathology treatment in Russian Federation, as the current practics and clinical guidelines on pediatric PH in Europe, and the recent trials published.

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