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<article article-type="research-article" dtd-version="1.3" xmlns:mml="http://www.w3.org/1998/Math/MathML" xmlns:xlink="http://www.w3.org/1999/xlink" xmlns:xsi="http://www.w3.org/2001/XMLSchema-instance" xml:lang="ru"><front><journal-meta><journal-id journal-id-type="publisher-id">cardio</journal-id><journal-title-group><journal-title xml:lang="ru">Кардиология</journal-title><trans-title-group xml:lang="en"><trans-title>Kardiologiia</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">0022-9040</issn><issn pub-type="epub">2412-5660</issn><publisher><publisher-name>Kardiomag</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.18087/cardio.2024.12.n2784</article-id><article-id custom-type="elpub" pub-id-type="custom">cardio-2784</article-id><article-categories><subj-group subj-group-type="heading"><subject>Research Article</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>ОБЗОРЫ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>REVIEWS</subject></subj-group></article-categories><title-group><article-title>Медикаментозное лечение хронической тромбоэмболической легочной гипертензии</article-title><trans-title-group xml:lang="en"><trans-title>Chronic Thromboembolic Pulmonary Hypertension Drug Treatment</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Иванов</surname><given-names>С. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Ivanov</surname><given-names>S. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Врач-детский кардиолог    Консультативно-диагностическое отделение № 1, д.м.н., ведущий научный сотрудник</p><p>Новосибирск, Россия</p></bio><bio xml:lang="en"><p>Pediatric cardiologist Consultative and diagnostic department No. 1</p><p>Novosibirsk, Russia</p></bio><email xlink:type="simple">ivanov0557@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Чернявский</surname><given-names>А. М.</given-names></name><name name-style="western" xml:lang="en"><surname>Chernyavsky</surname><given-names>A. M.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Генеральный директор ФГБУ «НМИЦ им. ак. Е.Н. Мешалкина» Минздрава России,  член-корр. РАН, д.м.н., профессор</p><p>Новосибирск, Россия</p></bio><bio xml:lang="en"><p>Director General, Corresponding Member of the Russian Academy of Sciences, Doctor of Medical Sciences, Professor</p><p>Novosibirsk, Russia</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Едемский</surname><given-names>А. Г.</given-names></name><name name-style="western" xml:lang="en"><surname>Edemsky</surname><given-names>A. G.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Врач-сердечно-сосудистый хирург,  Кардиохирургическое отделение № 2 ,  ФГБУ «НМИЦ им. ак. Е.Н. Мешалкина» Минздрава России, к.м.н.</p><p>Новосибирск, Россия</p></bio><bio xml:lang="en"><p>Cardiovascular surgeon Cardiac surgery department No. 2</p><p>PhD</p><p>Novosibirsk, Russia</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Васильцева</surname><given-names>О. Я.</given-names></name><name name-style="western" xml:lang="en"><surname>Vasiltseva</surname><given-names>O. Ya.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Ведущий научный сотрудник, Научно-исследовательский отдел хирургии аорты, коронарных и периферических артерий института  патологии кровообращения , ФГБУ «НМИЦ им. ак. Е.Н. Мешалкина» Минздрава России,   д.м.н.</p><p>Новосибирск, Россия</p></bio><bio xml:lang="en"><p>Leading researcher Research Department of Aortic Surgery, Coronary and Peripheral Arteries of the Institute of Circulatory PathologyDoctor of Medical Sciences.</p><p>Novosibirsk, Russia</p></bio><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБУ «Национальный медицинский исследовательский центр им. академика Е. Н. Мешалкина» Минздрава РФ</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Meshalkin National Medical Research Center</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2024</year></pub-date><pub-date pub-type="epub"><day>25</day><month>12</month><year>2024</year></pub-date><volume>64</volume><issue>12</issue><fpage>77</fpage><lpage>85</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Kardiomag, 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Kardiomag</copyright-holder><copyright-holder xml:lang="en">Kardiomag</copyright-holder><license xlink:href="https://cardio.elpub.ru/jour/about/submissions#copyrightNotice" xlink:type="simple"><license-p>https://cardio.elpub.ru/jour/about/submissions#copyrightNotice</license-p></license></permissions><self-uri xlink:href="https://cardio.elpub.ru/jour/article/view/2784">https://cardio.elpub.ru/jour/article/view/2784</self-uri><abstract><p>Основным методом лечения пациентов с хронической тромбоэмболической легочной гипертензией (ХТЭЛГ) является радикальное хирургическое вмешательство – легочная тромбэндартерэктомия (ЛЭЭ). Однако около 40% больных с ХТЭЛГ неоперабельны в связи с дистальным поражением легочного сосудистого русла или тяжестью гемодинамических нарушений. Почти у 30% пациентов с ХТЭЛГ после хирургического вмешательства отмечается персистирующая или рецидивирующая легочная гипертензия, требующая медикаментозного лечения ЛАГ-специфическими препаратами. В настоящем обзоре приведены актуальные данные по применению таргетной терапии у пациентов с ХТЭЛГ. Обсуждаются место, показания и доказательная база по применению основных групп специфических препаратов, в том числе стимуляторов растворимой гуанилатциклазы, ингибиторов фосфодиэстеразы пятого типа, антагонистов рецепторов эндотелина, аналогов простациклина.</p><p> </p></abstract><trans-abstract xml:lang="en"><p>The main treatment of patients with chronic thromboembolic pulmonary hypertension (CTEPH) is radical surgery, pulmonary thromboendarterectomy (PEA). However, about 40% of patients with CTEPH are inoperable due to distal pulmonary vascular lesions or the severity of hemodynamic disorders. Almost 30% of patients with CTEPH experience persistent or recurrent pulmonary hypertension after surgery, that requires a drug treatment with PAH-specific drugs. This review presents current data on the use of targeted therapy in patients with CTEPH. The review addresses the place, indications, and the evidence base for using the main groups of specific drugs, including stimulators of soluble guanylate cyclase, phosphodiesterase type 5 inhibitors, endothelin receptor antagonists, and prostacyclin analogues.</p><p> </p></trans-abstract><kwd-group xml:lang="ru"><kwd>Хроническая тромбоэмболическая легочная гипертензия</kwd><kwd>ЛАГ-специфическая терапия</kwd><kwd>риоцигуат</kwd></kwd-group><kwd-group xml:lang="en"><kwd>Chronic thromboembolic pulmonary hypertension</kwd><kwd>PAH-specific therapy</kwd><kwd>riociguat</kwd></kwd-group><funding-group><funding-statement xml:lang="ru">Конфликт интересов не заявлен</funding-statement><funding-statement xml:lang="en">No conflict of interest is reported.</funding-statement></funding-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">Ende-Verhaar YM, Cannegieter SC, Vonk Noordegraaf A, Delcroix M, Pruszczyk P, Mairuhu ATA et al. 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