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<article 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" xmlns:ali="http://www.niso.org/schemas/ali/1.0/" article-type="research-article" dtd-version="1.2" xml:lang="en"><front><journal-meta><journal-id journal-id-type="publisher-id">Systemic Hypertension</journal-id><journal-title-group><journal-title xml:lang="en">Systemic Hypertension</journal-title><trans-title-group xml:lang="ru"><trans-title>Системные гипертензии</trans-title></trans-title-group></journal-title-group><issn publication-format="print">2075-082X</issn><issn publication-format="electronic">2542-2189</issn><publisher><publisher-name xml:lang="en">Intermedservice Ltd</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="publisher-id">29613</article-id><article-id pub-id-type="doi">10.26442/2075-082X_2018.2.72-76</article-id><article-categories><subj-group subj-group-type="toc-heading" xml:lang="en"><subject>Articles</subject></subj-group><subj-group subj-group-type="toc-heading" xml:lang="ru"><subject>Статьи</subject></subj-group><subj-group subj-group-type="article-type"><subject>Research Article</subject></subj-group></article-categories><title-group><article-title xml:lang="en">Nitrogen oxide biochemical pathway in pulmonary arterial hypertension therapy and REPLACE trial results</article-title><trans-title-group xml:lang="ru"><trans-title>Биохимический путь оксида азота в терапии легочной артериальной гипертензии и результаты исследования RESPITE</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Shmalts</surname><given-names>A A</given-names></name><name xml:lang="ru"><surname>Шмальц</surname><given-names>Антон Алексеевич</given-names></name></name-alternatives><bio xml:lang="ru"><p>д-р мед. наук, вед. науч. сотр. отд-ния хирургического лечения заболеваний сердца с прогрессирующей легочной гипертензией, доц. каф. сердечно-сосудистой хирургии</p></bio><email>shmaltzanton@inbox.ru</email><xref ref-type="aff" rid="aff1"/><xref ref-type="aff" rid="aff4"/></contrib><contrib contrib-type="author"><name-alternatives><name xml:lang="en"><surname>Gorbachevsky</surname><given-names>S V</given-names></name><name xml:lang="ru"><surname>Горбачевский</surname><given-names>Сергей Валерьевич</given-names></name></name-alternatives><bio xml:lang="ru"><p>д-р мед. наук, проф., зав. отд-нием хирургического лечения заболеваний сердца с прогрессирующей легочной гипертензией, проф. каф. сердечно-сосудистой хирургии</p></bio><email>svgorby@newmail.ru</email><xref ref-type="aff" rid="aff3"/><xref ref-type="aff" rid="aff4"/></contrib></contrib-group><aff-alternatives id="aff1"><aff><institution xml:lang="en">A.N.Bakulev National Medical Research Center of Cardiovascular Surgery of the Ministry of Health of the Russian Federation</institution></aff><aff><institution xml:lang="ru">ФГБУ «НМИЦ ССХ им. А.Н.Бакулева», ФГБОУ ДПО РМАНПО</institution></aff></aff-alternatives><aff-alternatives id="aff2"><aff><institution xml:lang="en">Russian Medical Academy of Continuous Professional Education of the Ministry of Health of the Russian Federation</institution></aff><aff><institution xml:lang="ru">ФГБУ «НМИЦ ССХ им. А.Н.Бакулева», ФГБОУ ДПО РМАНПО</institution></aff></aff-alternatives><aff id="aff3"><institution>A.N.Bakulev National Medical Research Center of Cardiovascular Surgery of the Ministry of Health of the Russian Federation</institution></aff><aff id="aff4"><institution>Russian Medical Academy of Continuous Professional Education of the Ministry of Health of the Russian Federation</institution></aff><pub-date date-type="pub" iso-8601-date="2018-06-15" publication-format="electronic"><day>15</day><month>06</month><year>2018</year></pub-date><volume>15</volume><issue>2</issue><issue-title xml:lang="en">VOL 15, NO2 (2018)</issue-title><issue-title xml:lang="ru">ТОМ 15, №2 (2018)</issue-title><fpage>72</fpage><lpage>76</lpage><history><date date-type="received" iso-8601-date="2020-04-09"><day>09</day><month>04</month><year>2020</year></date></history><permissions><copyright-statement xml:lang="en">Copyright ©; 2018, Consilium Medicum</copyright-statement><copyright-statement xml:lang="ru">Copyright ©; 2018, ООО "Консилиум Медикум"</copyright-statement><copyright-year>2018</copyright-year><copyright-holder xml:lang="en">Consilium Medicum</copyright-holder><copyright-holder xml:lang="ru">ООО "Консилиум Медикум"</copyright-holder><ali:free_to_read xmlns:ali="http://www.niso.org/schemas/ali/1.0/"/><license><ali:license_ref xmlns:ali="http://www.niso.org/schemas/ali/1.0/">https://creativecommons.org/licenses/by-nc-sa/4.0</ali:license_ref></license></permissions><self-uri xlink:href="https://journals.eco-vector.com/2075-082X/article/view/29613">https://journals.eco-vector.com/2075-082X/article/view/29613</self-uri><abstract xml:lang="en"><p>Endogen nitric oxide (NO) and cyclic guanosine monophosphate (cGMP) deficiency in pulmonary vessels walls plays essential role in pulmonary arterial hypertension (PAH) pathogenesis. Soluble guanylate cyclase stimulator riociguat and phosphodiesterase-5 (PDE5) inhibitor sildenafil increase cGMP content and have proven clinical efficacy in PAH treatment. The potentially beneficial mechanisms of riociguat mechanism of action include endogen NO independence in cGMP synthesis and its independence from other phosphodiesterase isoferments (other than PDE5). Clinical options, safety and effectiveness of iPDE5 - riociguat transition in patients with PAH were for the first time shown in non-controlled study RESPITE and the assessment is continued in randomized placebo-controlled trial REPLACE.</p></abstract><trans-abstract xml:lang="ru"><p>Недостаточное содержание эндогенного оксида азота и циклического гуанозинмонофосфата (цГМФ) в стенке легочных сосудов играет важную роль в патогенезе легочной артериальной гипертензии (ЛАГ). Стимулятор растворимой гуанилатциклазы риоцигуат и ингибитор фосфодиэстеразы-5 (иФДЭ-5) силденафил увеличивают содержание цГМФ и имеют доказанную клиническую эффективность при ЛАГ. Потенциально выгодными особенностями механизма действия риоцигуата являются независимость от эндогенного оксида азота при синтезе цГМФ и независимость от других (помимо ФДЭ-5) изоферментов фосфодиэстераз. Клиническая возможность, безопасность и эффективность замены иФДЭ-5 на риоцигуат у больных ЛАГ впервые показана в неконтролируемом исследовании RESPITE и продолжает оцениваться в рандомизированном плацебо-контролируемом исследовании REPLACE.</p></trans-abstract><kwd-group xml:lang="en"><kwd>pulmonary arterial hypertension</kwd><kwd>PAH-specific therapy</kwd><kwd>soluble guanylate cyclase stimulators</kwd><kwd>phosphodiesterase-5 inhibitors</kwd><kwd>nitrogen oxide</kwd></kwd-group><kwd-group xml:lang="ru"><kwd>легочная артериальная гипертензия</kwd><kwd>ЛАГ-специфическая терапия</kwd><kwd>стимуляторы растворимой гуанилатциклазы</kwd><kwd>ингибиторы фосфодиэстеразы-5</kwd><kwd>оксид азота</kwd></kwd-group></article-meta></front><body></body><back><ref-list><ref id="B1"><label>1.</label><mixed-citation>Легочная гипертензия. Под ред. И.Е.Чазовой, Т.В.Мартынюк. М.: Практика, 2015. 2. Легочная гипертензия. 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