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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">pulmo</journal-id><journal-title-group><journal-title xml:lang="ru">Пульмонология</journal-title><trans-title-group xml:lang="en"><trans-title>PULMONOLOGIYA</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">0869-0189</issn><issn pub-type="epub">2541-9617</issn><publisher><publisher-name>Scientific and Practical Journal “PULMONOLOGIYA” LLC</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.18093/0869-0189-2020-30-4-427-436</article-id><article-id custom-type="elpub" pub-id-type="custom">pulmo-2149</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>ОРИГИНАЛЬНЫЕ ИССЛЕДОВАНИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>ORIGINAL STUDIES</subject></subj-group></article-categories><title-group><article-title>«Портрет» пациентов с идиопатической легочной гипертензией и хронической тромбоэмболической легочной гипертензией в зависимости от коморбидного статуса: особенности течения заболевания и прогноз</article-title><trans-title-group xml:lang="en"><trans-title>“Portrait” of patients with idiopathic pulmonary hypertension and chronic thromboembolic pulmonary hypertension depending on comorbid status: current and prognosis features</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>Таран</surname><given-names>И. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Taran</surname><given-names>I. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Таран Ирина Николаевна – аспирант отдела легочной гипертензии и заболеваний сердца Научно-исследовательского института клинической кардиологии им. А.Л.Мясникова Федерального государственного бюджетного учреждения «Национальный медицинский исследовательский центр кардиологии» Министерства здравоохранения Российской Федерации, младший научный сотрудник лаборатории реабилитации отдела мультифокального атеросклероза Федерального государственного бюджетного научного учреждения «Научно-исследовательский институт комплексных проблем сердечно-сосудистых заболеваний»</p><p>121552, Москва, ул. 3-я Черепковская, 15А, </p><p>Кемерово, 650002, Кемерово, Сосновый бульвар, 6</p></bio><bio xml:lang="en"><p>Irina N. Taran, Postgraduate student, Division of Pulmonary Hypertension and Cardiac Diseases, A.L.Myasnikov Research Institute of Clinical Cardiology, National Medical Research Center for Cardiology, Healthcare Ministry of Russia; Junior Researcher, Laboratory of Rehabilitation, Division of Multifocal Atherosclerosis, Federal Research Institute of Complex Issues of Cardiovascular Diseases, Siberian Department of Russian Academy of Science</p><p>ul. 3-ya Cherepkovskaya 15A, Moscow, 121552, </p><p>Sosnovyy bul'var 6, Kemerovo, 650002</p></bio><email xlink:type="simple">zaviirina@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>Белевская</surname><given-names>А. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Belevskaya</surname><given-names>A. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Белевская Анна Андреевна – кандидат медицинских наук, младший научный сотрудник отдела ультразвуковых методов исследования</p><p>121552, Москва, ул. 3-я Черепковская, 15А</p></bio><bio xml:lang="en"><p>Anna A. Belevskaya, Candidate of Medicine, Junior Researcher, Division of ultrasonic diagnostic techniques of Scientific research institute of clinical cardiology</p><p>ul. 3-ya Cherepkovskaya 15A, Moscow, 121552</p></bio><email xlink:type="simple">annabelevskaya@gmail.com</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Валиева</surname><given-names>З. С.</given-names></name><name name-style="western" xml:lang="en"><surname>Valieva</surname><given-names>Z. S.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Валиева Зарина Солтановна – кандидат медицинских наук, научный сотрудник отдела легочной гипертензии и заболеваний сердца</p><p>121552, Москва, ул. 3-я Черепковская, 15А</p></bio><bio xml:lang="en"><p>Zarina S. Valieva, Candidate of Medicine, Researcher, Division of Pulmonary Hypertension and Cardiac Diseases</p><p>ul. 3-ya Cherepkovskaya 15A, Moscow, 121552</p></bio><email xlink:type="simple">v.zarina.v@gmail.com</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Саидова</surname><given-names>М. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Saidova</surname><given-names>M. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Саидова Марина Абдулатиповна – доктор медицинских наук, профессор, руководитель отдела ультразвуковых методов исследования</p><p>121552, Москва, ул. 3-я Черепковская, 15А</p></bio><bio xml:lang="en"><p>Marina A. Saidova, Doctor of Medicine, Professor, Head of the Division of ultrasonic diagnostic techniques of Scientific research institute of clinical cardiology</p><p>ul. 3-ya Cherepkovskaya 15A, Moscow, 121552</p></bio><email xlink:type="simple">m.saidova@gmail.com</email><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Мартынюк</surname><given-names>Т. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Martynyuk</surname><given-names>T. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Мартынюк Тамила Витальевна – доктор медицинских наук, руководитель отдела легочной гипертензии и заболеваний сердца Научно-исследовательского института клинической кардиологии им. А.Л.Мясникова Федерального государственного бюджетного учреждения «Национальный медицинский исследовательский центр кардиологии» Министерства здравоохранения Российской Федерации, профессор кафедры кардиологии факультета дополнительного профессионального образования Федерального государственного бюджетного образовательного учреждения высшего образования «Российский национальный исследовательский медицинский университет имени Н.И.Пирогова» Министерства здравоохранения Российской Федерации</p><p>121552, Москва, ул. 3-я Черепковская, 15А, </p><p>117997, Москва, ул. Островитянова, 1</p></bio><bio xml:lang="en"><p>Tamila V. Martynyuk, Doctor of Medicine, Head of Division of Pulmonary Hypertension and Cardiac Diseases, A.L. Myasnikov Research Institute of Clinical Cardiology, National Medical Research Center for Cardiology, Healthcare Ministry of Russia; Professor, Department of Cardiology, Faculty of Postgraduate Medical Training, N.I.Pirogov Federal Russian State National Research Medical University, Healthcare Ministry of Russia</p><p>ul. 3-ya Cherepkovskaya 15A, Moscow, 121552, </p><p>ul. Ostrovityanova 1, Moscow, 117997</p></bio><email xlink:type="simple">trukhiniv@mail.ru</email><xref ref-type="aff" rid="aff-3"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Научно-исследовательский институт клинической кардиологии им. А.Л.Мясникова Федерального государственного бюджетного учреждения «Национальный медицинский исследовательский центр кардиологии» Министерства здравоохранения Российской Федерации;&#13;
Федеральное государственное бюджетное научное учреждение «Научно-исследовательский институт комплексных проблем сердечно-сосудистых заболеваний»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>A.L.Myasnikov Research Institute of Clinical Cardiology, National Medical Research Center for Cardiology, Healthcare Ministry of Russia;&#13;
Federal Research Institute of Complex Issues of Cardiovascular Diseases, Siberian Department of Russian Academy of Science</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Научно-исследовательский институт клинической кардиологии им. А.Л.Мясникова Федерального государственного бюджетного учреждения «Национальный медицинский исследовательский центр кардиологии» Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>A.L.Myasnikov Research Institute of Clinical Cardiology, National Medical Research Center for Cardiology, Healthcare Ministry of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-3"><aff xml:lang="ru"><institution>Научно-исследовательский институт клинической кардиологии им. А.Л.Мясникова Федерального государственного бюджетного учреждения «Национальный медицинский исследовательский центр кардиологии» Министерства здравоохранения Российской Федерации;&#13;
Федеральное государственное бюджетное образовательное учреждение высшего образования «Российский национальный исследовательский медицинский университет имени Н.И.Пирогова» Министерства здравоохранения Российской Федерации</institution><country>Россия</country></aff><aff xml:lang="en"><institution>A.L.Myasnikov Research Institute of Clinical Cardiology, National Medical Research Center for Cardiology, Healthcare Ministry of Russia;&#13;
N.I.Pirogov Federal Russian State National Research Medical University, Healthcare Ministry of Russia</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2020</year></pub-date><pub-date pub-type="epub"><day>17</day><month>10</month><year>2020</year></pub-date><volume>30</volume><issue>4</issue><fpage>427</fpage><lpage>436</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Таран И.Н., Белевская А.А., Валиева З.С., Саидова М.А., Мартынюк Т.В., 2020</copyright-statement><copyright-year>2020</copyright-year><copyright-holder xml:lang="ru">Таран И.Н., Белевская А.А., Валиева З.С., Саидова М.А., Мартынюк Т.В.</copyright-holder><copyright-holder xml:lang="en">Taran I.N., Belevskaya A.A., Valieva Z.S., Saidova M.A., Martynyuk T.V.</copyright-holder><license xml:lang="ru" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>Данная работа распространяется под лицензией Creative Commons Attribution 4.0.</license-p></license><license xml:lang="en" license-type="creative-commons-attribution" xlink:href="https://creativecommons.org/licenses/by/4.0/" xlink:type="simple"><license-p>This work is licensed under a Creative Commons Attribution 4.0 License.</license-p></license></permissions><self-uri xlink:href="https://journal.pulmonology.ru/pulm/article/view/2149">https://journal.pulmonology.ru/pulm/article/view/2149</self-uri><abstract><p>Изменение «портрета» пациентов с идиопатической легочной гипертензией (ИЛГ) и хронической тромбоэмболической легочной гипертензией (ХТЭ ЛГ) за последнее десятилетие предполагает необходимость разработки моделей фенотипов у больных данной когорты.</p><p>Целью исследования явилась оценка характера течения заболевания и прогноз пациентов с ИЛГ и неоперабельной ХТЭ ЛГ на основании формирования фенотипических групп в зависимости от коморбидного статуса.</p><sec><title>Материалы и методы</title><p>Материалы и методы. В исследование включены пациенты с ИЛГ (n = 88) и неоперабельной ХТЭ ЛГ (n = 38) в возрасте 38,5 [28,5; 51] и 53,5 [41; 58] года соответственно. На момент верификации диагноза и через 13 [12; 20] мес. выполнялись 6-минутный шаговый тест (6-МШТ) и спировелоэргометрия, трансторакальная эхокардиография (ЭхоКГ), катетеризации правых отделов сердца. Все пациенты были разделены на 5 групп согласно коморбидному статуcу: 1-я группа (n = 29) – лица с ИЛГ / ХТЭ ЛГ без коморбидной патологии; 2-я группа (n = 23) – с индексом массы тела (ИМТ) ≥ 25 кг / м2; 3-я (n = 27) группа – с ИМТ ≥ 25 кг / м2 и дислипидемией; 4-я группа (n = 30) – с гипертонической болезнью, ИМТ ≥ 25 кг / м2 и дислипидемией; 5-я группа (n = 17) – с перечисленными коморбидными патологиями и нарушениями углеводного обмена (НУО).</p></sec><sec><title>Результаты</title><p>Результаты. Исходно достоверно более тяжелый функциональный статус наблюдался у больных 4-й и 5-й групп по сравнению с лицами, распределенными в 1-ю и 3-ю группы. Согласно данным 3-мерной ЭхоКГ, у пациентов 4-й и 5-й групп выявлено достоверно более выраженное увеличение объемов правого желудочка по сравнению с таковыми показателями у больных 1-й и 3-й групп. При ИЛГ в 1-й и 3-й группах достоверно более часто отмечались положительные результаты теста на вазореактивность (34,5 и 48,2 %) по сравнению с 4-й (6,7 %) и 5-й (5,9 %) группами. Период с момента верификации диагноза до добавления 2-го специфического препарата был значимо короче у пациентов с ИЛГ / ХТЭ ЛГ 5-й группы по сравнению с больными 3-й группы. При ИЛГ в 1-й и 3-й группах достоверно реже отмечался высокий риск летального исхода через 13 [12; 30] мес. лечения по сравнению с таковым показателем в 5-й группе.</p></sec><sec><title>Заключение</title><p>Заключение. Сочетание ИМТ ≥ 25 кг / м2 , дислипидемии, гипертонической болезни, как и дополнительное наличие НУО, ассоциируется с менее благоприятным течением заболевания у пациентов с ИЛГ и неоперабельной ХТЭ ЛГ.</p></sec></abstract><trans-abstract xml:lang="en"><p>Changing the "portrait" of patients with idiopathic pulmonary hypertension (IPH) and chronic thromboembolic pulmonary hypertension (CTPH) over the past decade suggests the need to develop models of phenotypes in patients with this cohort.</p><p>The purpose of the study was to estimate the nature of the disease course and prognosis of patients with IPH and inoperable CTPH based on the formation of phenotypic groups depending on the comorbid status.</p><sec><title>Materials and Methods</title><p>Materials and Methods. Patients with IPH (n = 88) and inoperable CTPH (n = 38) aged 38.5 [28.5; 51] and 53.5 [41; 58] years respectively were enrolled in the study. 6-minute step test (6-MST) and spiroveloergometry, transthoracic echocardiography (EchoCG), catheterization of the right side of the heart was performed at the moment of diagnosis verification and in 13 [12; 20] months. All patients were divided into 5 groups according to comorbid status: Group 1 (n = 29) – patients with IPH/CTPH without comorbid pathology; Group 2 (n = 23) with body mass index (BMI) ≥ 25 kg/m2 ; Group 3 (n = 27) – with BMI ≥ 25 kg/m2 and dyslipidemia; Group 4 (n = 30) with hypertension, BMI ≥ 25 kg/m2 and dyslipidemia; Group 5 (n = 17) with the listed comorbid pathologies and carbohydrate metabolic disorders.</p></sec><sec><title>Results</title><p>Results. Initially more severe functional status was observed in patients of the 4th and 5th groups as compared to those in the 1st and 3rd groups. According to the data of the 3-dimensional EchoCG, in patients of the 4th and 5th groups a reliably more expressed increase in the volume of the right ventricle was revealed in comparison with such values in the 1st and 3rd groups. In IPH groups 1 and 3, the positive results of the vasoreactivity test (34.5% and 48.2%) were significantly more frequent in comparison with groups 4 (6.7%) and 5 (5.9%). The period from the diagnosis verification to adding the 2nd specific drug was significantly shorter in patients with IPH/CTPH of the 5th group compared to patients of the 3rd group. a high risk of lethal outcome after 13 [12; 30] months of treatment was significantly less frequently observed with IPH in Groups 1 and 3 as compared to Group 5.</p></sec><sec><title>Conclusion</title><p>Conclusion. The combination of BMI ≥ 25 kg/m2, dyslipidemia, hypertension, as well as the additional presence of carbohydrate metabolism disorders is associated with a less favorable course of the disease in patients with IPH and inoperable CTPH.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>идиопатическая легочная гипертензия</kwd><kwd>неоперабельная хроническая тромбоэмболическая легочная гипертензия</kwd><kwd>фенотипы</kwd><kwd>коморбидная патология</kwd><kwd>прогноз</kwd></kwd-group><kwd-group xml:lang="en"><kwd>idiopathic pulmonary hypertension</kwd><kwd>inoperable chronic thromboembolic pulmonary hypertension</kwd><kwd>phenotypes</kwd><kwd>comorbid pathology</kwd><kwd>prognosis</kwd></kwd-group></article-meta></front><back><ref-list><title>References</title><ref id="cit1"><label>1</label><citation-alternatives><mixed-citation xml:lang="ru">D'Alonzo G.E., Barst R.J., Ayres S.M. et al. Survival in patients with primary pulmonary hypertension. 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