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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">cardiotomsk</journal-id><journal-title-group><journal-title xml:lang="ru">Сибирский журнал клинической и экспериментальной медицины</journal-title><trans-title-group xml:lang="en"><trans-title>Siberian Journal of Clinical and Experimental Medicine</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">2713-2927</issn><issn pub-type="epub">2713-265X</issn><publisher><publisher-name>TSU publishing</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.29001/2073-8552-2019-34-2-54-62</article-id><article-id custom-type="elpub" pub-id-type="custom">cardiotomsk-761</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>КЛИНИЧЕСКИЕ ИССЛЕДОВАНИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>CLINICAL STUDIES</subject></subj-group></article-categories><title-group><article-title>ПОКАЗАНИЯ К ИМПЛАНТАЦИИ И ЭФФЕКТИВНОСТЬ ИСПОЛЬЗОВАНИЯ КАРДИОВЕРТЕРОВ-ДЕФИБРИЛЛЯТОРОВ У БОЛЬНЫХ С ДИЛАТАЦИОННОЙ КАРДИОМИОПАТИЕЙ</article-title><trans-title-group xml:lang="en"><trans-title>INDICATIONS FOR IMPLANTATION AND EFFICIENCY OF CARDIOVERTER-DEFIBRILLATORS IN PATIENTS WITH DILATED CARDIOMYOPATHY</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-2651-2111</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Соловьева</surname><given-names>Е. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Solovyeva</surname><given-names>E. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>аспирант кафедры факультетской терапии № 1 лечебного факультета</p><p>119991, Российская Федерация, Москва, ул. Трубецкая, 8, стр. 2</p></bio><bio xml:lang="en"><p>Graduate Student, Department of Faculty Therapy No. 1, Medical Faculty</p><p>8/2, Trubetskaya str., Moscow, 119991, Russian Federation</p></bio><email xlink:type="simple">funtova.2014@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/0000-0002-5253-793X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Благова</surname><given-names>О. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Blagova</surname><given-names>O. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д-р мед. наук, профессор кафедры факультетской терапии № 1 лечебного факультета</p><p>119991, Российская Федерация, Москва, ул. Трубецкая, 8, стр. 2</p></bio><bio xml:lang="en"><p>Dr. Sci. (Med.), Professor, Department of Faculty Therapy № 1, Medical Faculty</p><p>8/2, Trubetskaya str., Moscow, 119991, Russian Federation</p></bio><email xlink:type="simple">blagovao@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/0000-0003-2326-9347</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Седов</surname><given-names>В. П.</given-names></name><name name-style="western" xml:lang="en"><surname>Sedov</surname><given-names>V. P.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д-р мед. наук, профессор кафедры лучевой диагностики</p><p>119991, Российская Федерация, Москва, ул. Трубецкая, 8, стр. 2</p></bio><bio xml:lang="en"><p>Dr. Sci. (Med.), Professor, Head of the Department of Radiology</p><p>8/2, Trubetskaya str., Moscow, 119991, Russian Federation</p></bio><email xlink:type="simple">vps52@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/0000-0002-4197-9732</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Коган</surname><given-names>Е. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Kogan</surname><given-names>E. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д-р мед. наук, профессор кафедры патологической анатомии</p><p>119991, Российская Федерация, Москва, ул. Трубецкая, 8, стр. 2</p></bio><bio xml:lang="en"><p>Dr. Sci. (Med.), Professor, Head of the Department of Pathological Anatomy</p><p>8/2, Trubetskaya str., Moscow, 119991, Russian Federation</p></bio><email xlink:type="simple">koganevg@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/0000-0002-6244-9546</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Заклязьминская</surname><given-names>Е. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Zaklyazminskaya</surname><given-names>E. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д-р мед. наук, профессор, заведующая лабораторией медицинской генетики</p><p>119991, Российская Федерация, Москва, Абрикосовский пер., 2</p></bio><bio xml:lang="en"><p>Dr. Sci. (Med.), Professor, Head of the Medical Genetics Laboratory</p><p>2, Abrikosovsky Lane, Moscow, 119991, Russian Federation</p></bio><email xlink:type="simple">helenezak@gmail.com</email><xref ref-type="aff" rid="aff-2"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Первый Московский государственный медицинский университет имени И.М. Сеченова Министерства здравоохранения Российской Федерации (Сеченовский университет)</institution><country>Россия</country></aff><aff xml:lang="en"><institution>I.M. Sechenov First Moscow State Medical University</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>Российский научный центр хирургии имени академика Б.В. Петровского</institution><country>Россия</country></aff><aff xml:lang="en"><institution>Petrovsky National Research Center of Surgery</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2019</year></pub-date><pub-date pub-type="epub"><day>09</day><month>07</month><year>2019</year></pub-date><volume>34</volume><issue>2</issue><fpage>54</fpage><lpage>62</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Соловьева Е.А., Благова О.В., Седов В.П., Коган Е.А., Заклязьминская Е.В., 2019</copyright-statement><copyright-year>2019</copyright-year><copyright-holder xml:lang="ru">Соловьева Е.А., Благова О.В., Седов В.П., Коган Е.А., Заклязьминская Е.В.</copyright-holder><copyright-holder xml:lang="en">Solovyeva E.A., Blagova O.V., Sedov V.P., Kogan E.A., Zaklyazminskaya E.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://www.sibjcem.ru/jour/article/view/761">https://www.sibjcem.ru/jour/article/view/761</self-uri><abstract><p>Цель: оценить частоту и предикторы оправданных срабатываний кардиовертеров-дефибрилляторов у больных с дилатационной кардиомиопатией (ДКМП), а также их влияние на общую и внезапную смертность.Материал и методы. Наблюдались 275 больных с синдромом ДКМП, средний возраст 46,8±12,5 лет, 185 мужчин и 90 женщин. Критериями включения были конечный диастолический размер (КДР) левого желудочка (ЛЖ) более 5,5 см и фракция выброса (ФВ) ЛЖ менее 50%. Из исследования исключались больные со стенозами коронарных артерий более 50%. Имплантируемые кардиовертеры-дефибрилляторы ИКД (n=44) и сердечные ресинхронизирующие устройства с функцией дефибриллятора/cardiac resynchronization therapy-defibrillator CRT-D (n=32) имплантированы 76 (27,6%) пациентам, в том числе 53 мужчинам и 23 женщинам, средний возраст 48,9±12,9 лет, КДР ЛЖ 6,7±0,8 см, ФВ ЛЖ 28,2±9,9%). В группу сравнения вошли 199 больных (72,4%) без устройств (132 мужчины и 67 женщин, средний возраст 46,0±12,3 лет, КДР ЛЖ 6,5±0,8 см, ФВ ЛЖ 32,0±10,2%). Срок наблюдения составил 27 [24; 30] мес.Результаты. Среди всех больных с синдромом ДКМП частота внезапной смерти составила 2,9%, летальность 18,9%, «смерть+трансплантация» 22,6%; среди пациентов с дефибрилляторами 2,6; 23,7; 32,9%, с ИКД 4,6; 22,7 и 34,1%, с CRT-D 0; 25,0 и 31,3%, у пациентов без устройств 3,0; 17,1; 18,6% соответственно. Значимо более высокие показатели «внезапная смерть+срабатывания» были отмечены у больных с дефибрилляторами (26,3 vs 3,0%, p&lt;0.001). Установлена преимущественно воспалительная (53,1%), первичная/генетическая (19,6%) и смешанная (25,1%) природа синдрома ДКМП. Генетические формы были представлены некомпактным миокардом, аритмогенной дисплазией правого желудочка (АДПЖ), миодистрофиями, амилоидозом. Выявлены мутации в генах LMNA (n=1), DES (n=2), DSP (n=2), EMD (n=2), PKP2 (n=1), TTR (n=1), MYH7+MyBPC3 (n=1), MyBPC3 (n=4). Частота оправданных шоков ИКД/CRT-D составила 23,7% (n=13/5). Единственным статистически значимым предиктором оправданных срабатываний оказалась первичная/генетическая природа ДКМП, выявленная у 100% больных с шоками (в сочетании с миокардитом в 77,8%/ изолированная в 22,2 vs 29,3/22,4% у пациентов без срабатываний), p&lt;0,001, AUC 0.747, ОР 1.66, 95% ДИ 0.711 до 3.885. Желудочковая тахикардия (ЖТ) зарегистрирована у 84% больных с шоками (17% устойчивая/67% неустойчивая vs 1,7/72% в группе без срабатываний), р=0,06. У больных с перенесенными шоками чаще фиксировалось снижение вольтажа комплекса QRS (39 vs 6,9%, р&lt;0,05), ФВ была статистически значимо выше (34,4±9,7 vs 25,9±8,8%, p&lt;0,005). Стоит отметить, что признаки гипертрофии ЛЖ у пациентов с шоками встречались в 22,4% случаев, в группе без шоков в 41,4% (р=0,07).Заключение. Основным предиктором оправданных срабатываний дефибрилляторов у больных с синдромом ДКМП является его первичная/генетическая природа, которую целесообразно использовать в качестве самостоятельного критерия отбора на имплантацию ИКД/CRT-D. Возраст, ФВ, функциональный класс (ФК) сердечной недостаточности прогностической значимости не имели. Дополнительными предикторами оправданных шоков явились устойчивая/неустойчивая ЖТ, низкий вольтаж QRS и отсутствие признаков гипертрофии ЛЖ на электрокардиограмме (ЭКГ). </p></abstract><trans-abstract xml:lang="en"><sec><title>Purpose</title><p>Purpose. To assess the frequency and predictors of appropriate shocks of cardioverter-defibrillators in patients with dilated cardiomyopathy (DCM) syndrome and the impact on the total mortality and sudden cardiac death (SCD).Material and Methods. A total of 275 patients with DCM syndrome (average age of 46.8 ± 12.5 years; 185 males and 90 females) were observed. Inclusion criteria were left ventricular (LV) end-diastolic diameter (EDD) more than 5.5 cm and LV ejection fraction (EF) less than 50%. Patients with coronary artery stenosis more than 50% were excluded. Implantable cardioverter-defibrillator (ICD) (n=44) and cardiac resynchronization therapy defibrillator (CRT-D) (n=32) were implanted in 76 (27.6%) patients (53 males and 23 females, average age of 48.9±12.9 years, LV EDD of 6.7±0.8 cm, and LV EF of 28.2±9.9%). A comparison group comprised 199 patients (72.4%) without devices (132 males and 67 females, average age of 46.0±12.3 years, LV EDD of 6.5±0.8 cm, and LV EF of 32.0±10.2%). The average follow-up was 27 (24; 30) months.Results. SCD in patients with DCM syndrome was recorded in 2.9% of cases; the total mortality rate was 18.9%; the rate of death + transplantation was 22.6%. The SCD, total mortality, and death+transplantation rates were 2.6% (4.6/0%), 23.7% (22.7/25.0%), and 32.9% (34.1/31.3%) in patients with devices (ICD/CRT-D) and 3.0%, 17.1%, and 16.6% in patients without devices, respectively. The rate of SCD+appropriate shocks (ASR) was significantly higher in study group: 26.3 vs 3.0% in comparison group (p&lt;0.001). The nature of DCM syndrome was predominantly inflammatory (53%), primary (genetic) (19.6%), and multifactorial (25.1%). Genetic forms of DCM were represented by non-compaction cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy (ARVC), myopathies, and amyloidosis. The pathogenic mutations in the genes LMNA (n=1), DES (n=2), DSP (n=2), EMD (n=2), PKP2 (n=1), TTR (n=1), MUH7+MyBPC3 (n=1), and MyBPC3 (n=4) were detected. The ASR (ICD/CRT-D) rate was 23.7% (n=13/5). The only reliable predictor of ASR was the generic nature of DCM syndrome, identified in 100% of patients with shocks (in the presence of myocarditis in 77.8%/isolated in 22.2%) in comparison with 51.7% (29.3/22.4%) in patients without ASR (p&lt;0.002, AUC 0.747, RR 1.66, 95% CI 0.711-3.885). Ventricular tachycardia (VT) was registered in 84% of patients with shocks (stable/unstable VT rates of 17/67%) vs 1.7/72%, in patients without shocks (р=0.06). In patients with shocks, low QRS voltage (39 vs 6.9%, р&lt;0.05) and the absence of LV hypertrophy signs on the ECG (77.6 vs 58.6%, р&gt;0.05) were registered more often. The average LV EF was higher in patients with ASR (34.4±9.7%) in comparison with patients without ASR (25.9±8.8%), р&lt;0.005. </p></sec><sec><title>Conclusions</title><p>Conclusions. The genetic nature of DCM syndrome is an important predictor of appropriate shocks and an independent selection criterion for ICD/CRT-D implantation. Age, NYHA class, and LV EF did not show prognostic significance. Additional predictors of appropriate shocks were sustained/unsustained VT, low QRS voltage, and the absence of LV hypertrophy signs on the ECG. </p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>кардиовертеры-дефибрилляторы</kwd><kwd>дилатационная кардиомиопатия</kwd><kwd>внезапная сердечная смерть</kwd><kwd>миокардит</kwd><kwd>желудочковая тахикардия</kwd><kwd>этиология</kwd></kwd-group><kwd-group xml:lang="en"><kwd>cardioverter-defibrillators</kwd><kwd>dilated cardiomyopathy</kwd><kwd>sudden cardiac death</kwd><kwd>myocarditis</kwd><kwd>ventricular tachycardia</kwd><kwd>etiology</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">Pasquale Losurdo, Davide Stolfo, Marco Merlo, Giulia Barbati, Marco Gobbo, Marta Gigli, et al. Early arrhythmic events in idiopathic dilated cardiomyopathy. 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