evrazkarЕвразийский Кардиологический ЖурналEurasian heart journal2225-16852305-0748Евразийская ассоциация кардиологов10.38109/2225-1685-2018-1-48-58evrazkar-275Research ArticleОБЗОРREVIEWОСОБЕННОСТИ ТАКТИКИ ВЕДЕНИЯ И ВЫБОРА СПЕЦИФИЧЕСКОЙ ТЕРАПИИ У ВЗРОСЛЫХ ПАЦИЕНТОВ С ЛЕГОЧНОЙ АРТЕРИАЛЬНОЙ ГИПЕРТЕНЗИЕЙ, АССОЦИИРОВАННОЙ С ВРОЖДЕННЫМИ ПОРОКАМИ СЕРДЦАTACTICS FEATURES OF CONDUCTING AND CHOOSING SPECIFIC THERAPY FOR ADULT PATIENTS WITH PULMONARY ARTERIAL HYPERTENSION ASSOCIATED WITH CONGENITAL HEART DISEASEАрхиповаО. А.ArkhipovaO. A.

к.м.н., научный сотрудник отдела легочной гипертензии и заболеваний сердца 

121552, г. Москва, 3-я Черепковская, д. 15 а.

Тел.: 8-495-414-68-33

PhD, Researcher of department of pulmonary hypertension and heart disease 

121552, Moscow, 3rd Street Cherepkovskaya, 15a

tel.: 8-495-414-68-33

Olga_ark@list.ruМартынюкТ. В.MartynyukT. V.

Д.м.н., руководитель отдела легочной гипертензии и заболеваний сердца 

121552, г. Москва, 3-я Черепковская, д. 15 а

Тел.: 8-495-414-64-50

MD, leading researcher, Head of the department of pulmonary hypertension and heart disease 

121552, Moscow, 3rd Street Cherepkovskaya, 15a

tel.: 8-495-414-64-50

noemail@neicon.ruЧазоваИ. Е.ChazovaI. Ye.

Академик РАН, профессор, директор института клинической кардиологии 

121552, г. Москва, 3-я Черепковская, д. 15 а

Тел.: 8-495-414-63-05

Academician of Russian Academy of Science, MD, Director 

121552, Moscow, 3rd Street Cherepkovskaya, 15a

tel.: 8-495-414-63-05

noemail@neicon.ruФГБУ «Национальный медицинский исследовательский центр кардиологии»Минздрава РоссииРоссияThe National Medical Research Centre of cardiology of Ministry of HealthRussian Federation201830032018014858Copyright © Архипова О.А., Мартынюк Т.В., Чазова И.Е., 20182018Архипова О.А., Мартынюк Т.В., Чазова И.Е.Arkhipova O.A., Martynyuk T.V., Chazova I.Y.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.heartj.asia/jour/article/view/275

ЛАГ, ассоциированная с ВПС, по данным Российского регистра легочной артериальной гипертензии (ЛАГ) и хронической тромбоэмболической легочной гипертензии (ХТЭЛГ), имеет высокую распространённость и занимает второе место после идиопатической легочной гипертензии (ИЛГ) по распространенности среди всех форм ЛАГ и первое место среди ассоциированных форм. При развитии ЛАГ пациенты с врожденными пороками сердца (ВПС) нуждаются в назначении постоянной ЛАГ-специфической терапии. В статье представлен обзор клинических исследований лекарственных препаратов, зарегистрированных в Российской Федерации в настоящее время в данной когорте больных, включающий антагонисты рецепторов эндотелина (бозентан, мацитентан, амбризентан), ингибиторы фосфодиэстеразы типа 5 (силденафил), стимуляторы растворимой гуанилатциклазы (риоцигуат) и аналоги простациклина (илопрост).

PAH associated with CHD according to the Russian Register of Pulmonary Arterial Hypertension (PAH) and Chronic Thromboembolic Pulmonary Hypertension (CTEPH) is highly prevalent and ranks second after idiopathic pulmonary hypertension (IPH) in terms of prevalence among all forms of PAH and the first among associated forms. With the development of PAH, patients with congenital heart disease (CHD) require the appointment of a constant PAH-specific therapy. The article presents an overview of clinical trials of medications currently registered in the Russian Federation for this cohort of patients, including endothelin receptor antagonists (bosentan, macitentan, ambrisentan), phosphodiesterase type 5 inhibitors (sildenafil), soluble guanylate cyclase stimulants (riociguat) and prostacyclin analogues (iloprost).

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