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<article article-type="review-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">kpccz</journal-id><journal-title-group><journal-title xml:lang="ru">Комплексные проблемы сердечно-сосудистых заболеваний</journal-title><trans-title-group xml:lang="en"><trans-title>Complex Issues of Cardiovascular Diseases</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2306-1278</issn><issn pub-type="epub">2587-9537</issn><publisher><publisher-name>Federal State Budgetary Institution “Research Institute for Complex Issues of Cardiovascular Diseases”</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.17802/2306-1278-2024-13-3-92-104</article-id><article-id custom-type="elpub" pub-id-type="custom">kpccz-1370</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>ОНЛАЙН. АНАЛИТИЧЕСКИЙ ОБЗОР.  Кардиология. Внутренние болезни</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>REVIEW. Cardiology. Internal medicine</subject></subj-group></article-categories><title-group><article-title>ТРАНСТИРЕТИНОВАЯ (ATTR) АМИЛОИДНАЯ КАРДИОМИОПАТИЯ В НАШИ ДНИ. РАСПРОСТРАНЕННОЕ ЗАБОЛЕВАНИЕ С ОТКРЫТЫМИ ПРОБЛЕМАМИ</article-title><trans-title-group xml:lang="en"><trans-title>TRANSTIRETINIC (ATTR) AMYLOID CARDIOMYOPATHY NOWADAYS. A COMMON DISEASE WITH OPEN PROBLEMS</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-3922-009X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Голубовская</surname><given-names>Дарья Петровна</given-names></name><name name-style="western" xml:lang="en"><surname>Golubovskaya</surname><given-names>Darya P.</given-names></name></name-alternatives><bio xml:lang="ru"><p>младший научный сотрудник лаборатории фиброгенеза миокарда федерального государственного бюджетного научного учреждения «Научно-исследовательский институт комплексных проблем сердечно-сосудистых заболеваний», Кемерово, Российская Федерация</p></bio><bio xml:lang="en"><p>Junior Researcher at the Laboratory of Myocardial Fibrogenesis, Federal State Budgetary Institution “Research Institute for Complex Issues of Cardiovascular Diseases”, Kemerovo, Russian Federation</p></bio><email xlink:type="simple">dasha250695k@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-4771-484X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Печерина</surname><given-names>Тамара Борзалиевна</given-names></name><name name-style="western" xml:lang="en"><surname>Pecherina</surname><given-names>Tamara B.</given-names></name></name-alternatives><bio xml:lang="ru"><p>доктор медицинских наук, доцент заведующая лабораторией фиброгенеза миокарда федерального государственного бюджетного научного учреждения «Научно-исследовательский институт комплексных проблем сердечно-сосудистых заболеваний», Кемерово, Российская Федерация</p></bio><bio xml:lang="en"><p>PhD, Associate Professor, Head of the Laboratory of Myocardial Fibrogenesis, Federal State Budgetary Institution “Research Institute for Complex Issues of Cardiovascular Diseases”, Kemerovo, Russian Federation</p></bio><email xlink:type="simple">tb.pechorina@gmail.com</email><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-4642-3610</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Барбараш</surname><given-names>Ольга Леонидовна</given-names></name><name name-style="western" xml:lang="en"><surname>Barbarash</surname><given-names>Olga L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>академик РАН, доктор медицинских наук, профессор директор федерального государственного бюджетного научного учреждения «Научно-исследовательский институт комплексных проблем сердечно-сосудистых заболеваний», Кемерово, Российская Федерация</p></bio><bio xml:lang="en"><p>Member of the Russian Academy of Sciences, Professor, PhD, Head of the Federal State Budgetary Institution “Research Institute for Complex Issues of Cardiovascular Diseases”, Kemerovo, Russian Federation</p></bio><email xlink:type="simple">olb61@mail.ru</email><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Федеральное государственное бюджетное научное учреждение «Научно-исследовательский институт комплексных проблем сердечно-сосудистых заболеваний»</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Federal State Budgetary Institution “Research Institute for Complex Issues of Cardiovascular Diseases”</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2024</year></pub-date><pub-date pub-type="epub"><day>30</day><month>09</month><year>2024</year></pub-date><volume>13</volume><issue>3</issue><fpage>92</fpage><lpage>104</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Голубовская Д.П., Печерина Т.Б., Барбараш О.Л., 2024</copyright-statement><copyright-year>2024</copyright-year><copyright-holder xml:lang="ru">Голубовская Д.П., Печерина Т.Б., Барбараш О.Л.</copyright-holder><copyright-holder xml:lang="en">Golubovskaya D.P., Pecherina T.B., Barbarash O.L.</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://www.nii-kpssz.com/jour/article/view/1370">https://www.nii-kpssz.com/jour/article/view/1370</self-uri><abstract><sec><title>Основные положения</title><p>Основные положения</p><p>Представлен обзор актуальных литературных данных, которые посвящены последним изменениям в эпидемиологии и особенностям транстиретинового амилоидоза. В обзоре освещены факторы, повышающие вероятность наличия у пациента данного заболевания, акцентировано внимание на группе больных высокого риска, а также представлены проблемы неинвазивного диагностического ведения лиц с амилоидозом сердца в клинической практике.</p></sec><sec><title> </title><p> </p></sec><sec><title>Резюме</title><p>Резюме</p><p>Транстиретиновая (ATTR) амилоидная кардиомиопатия – тяжелое инфильтративное заболевание сердца с формированием сердечной недостаточности (СН), как правило, с сохранной и умеренно сниженной фракцией выброса левого желудочка, с рестриктивным и ложногипертрофическим фенотипом, негативно влияющее на прогноз и качество жизни пациентов. В отсутствие своевременной специфической терапии медиана выживаемости приближена к 2–3,5 годам для разных форм ATTR-амилоидоза. Достижения в области неинвазивной визуализации сердца, доступность генетического тестирования,выделение групп пациентов высокого риска амилоидной кардиомиопатии, появление новых методов специфического лечения хоть и привели к увеличению выявляемости заболевания, однако уже на ее поздних стадиях (когда представлены развернутая картина СН и выраженное утолщение стенок сердца), что отрицательно сказывается на прогнозе больных. Междисциплинарный подход, настороженность и своевременная диагностика имеют решающее значение как для ATTR-амилоидоза, так и для проблемы СН в целом.</p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Highlights</title><p>Highlights</p><p>The article presents a review of literature data devoted to the latest changes in the epidemiology and features of transthyretin (ATTR) amyloidosis. The review highlights factors that increase the likelihood of a patient having this disease, emphasizing the group of high-risk patients, and discusses issues of non-invasive diagnostic management of a patient with cardiac amyloidosis in clinical practice.</p></sec><sec><title> </title><p> </p></sec><sec><title>Abstract</title><p>Abstract</p><p>Transthyretin (ATTR) amyloid cardiomyopathy is a severe infiltrative heart disease that followed by heart failure (HF) with a preserved and moderately reduced left ventricular ejection fraction and a restrictive phenotype. It negatively affects the prognosis and quality of life of patients. In the absence of timely treatment, the median survival is 2–3.5 years for different forms of ATTR amyloidosis. Advances in noninvasive cardiac imaging, availability of genetic testing, identification of «high risk» groups of patients with amyloid cardiomyopathy, and the emergence of new methods of treatment have increased the detection rate of the disease, but only at its later stages (when a patient suffers from heart failure and pronounced hypertrophic cardiomyopathy), which negatively affects the prognosis of patients. Therefore, an interdisciplinary approach, vigilance, and timely diagnostics are crucial for both ATTR amyloidosis and the problem of heart failure in general.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>Транстиретиновая амилоидная кардиомиопатия</kwd><kwd>Амилоидоз</kwd><kwd>Сердечная недостаточность с сохранной фракцией выброса</kwd><kwd>Сцинтиграфия</kwd></kwd-group><kwd-group xml:lang="en"><kwd>Transthyretin amyloid cardiomyopathy</kwd><kwd>Amyloidosis</kwd><kwd>Heart failure with preserved ejection fraction</kwd><kwd>Scintigraphy</kwd></kwd-group><funding-group><funding-statement xml:lang="ru">Авторы заявляют об отсутствии финансирования исследования.</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">Hassan W., Al-Sergani H., Mourad W., Tabbaa R. Amyloid heart disease. 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