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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="en"><front><journal-meta><journal-id journal-id-type="publisher-id">rpcardio</journal-id><journal-title-group><journal-title xml:lang="en">Rational Pharmacotherapy in Cardiology</journal-title><trans-title-group xml:lang="ru"><trans-title>Рациональная Фармакотерапия в Кардиологии</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1819-6446</issn><issn pub-type="epub">2225-3653</issn><publisher><publisher-name>«SILICEA-POLIGRAF» LLC</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.20996/1819-6446-2021-06-19</article-id><article-id custom-type="elpub" pub-id-type="custom">rpcardio-2514</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="en"><subject>CURRENT QUESTIONS OF CLINICAL PHARMACOLOGY</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="ru"><subject>АКТУАЛЬНЫЕ  ВОПРОСЫ  КЛИНИЧЕСКОЙ ФАРМАКОЛОГИИ</subject></subj-group></article-categories><title-group><article-title>Comparative Characteristics of Beta-Blockers in Patients with Congenital Long QT Syndrome</article-title><trans-title-group xml:lang="ru"><trans-title>Сравнительная характеристика бета-адреноблокаторов при лечении пациентов с врожденным синдромом удлиненного интервала QT</trans-title></trans-title-group></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0003-3938-3822</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Прошляков</surname><given-names>А. Ю.</given-names></name><name name-style="western" xml:lang="en"><surname>Proshlyakov</surname><given-names>A. Yu.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Прошляков Алексей Юрьевич</p></bio><bio xml:lang="en"><p>Alexey Y Proshlyakov</p></bio><email xlink:type="simple">a.proshlyakov96@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/0000-0003-1485-6072</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Чомахидзе</surname><given-names>П. Ш.</given-names></name><name name-style="western" xml:lang="en"><surname>Chomakhidze</surname><given-names>P. Sh.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Чомахидзе Пётр Шалвович – eLibrary SPIN 6230-5610</p></bio><bio xml:lang="en"><p>Peter Sh. Chomakhidze - eLibrary SPIN 6230-5610</p></bio><xref ref-type="aff" rid="aff-1"/></contrib><contrib contrib-type="author" corresp="yes"><contrib-id contrib-id-type="orcid">https://orcid.org/0000-0002-1645-781X</contrib-id><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Новикова</surname><given-names>Н. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Novikova</surname><given-names>N. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>Новикова Нина Александровна – eLibrary SPIN 7876-8609</p></bio><bio xml:lang="en"><p>Nina A. Novikova - eLibrary SPIN 7876-8609</p></bio><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>Первый Московский государственный медицинский университет им. И. М. Сеченова (Сеченовский Университет)</institution><country>Россия</country></aff><aff xml:lang="en"><institution>I.M. Sechenov First Moscow State Medical University (Sechenov University)</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2021</year></pub-date><pub-date pub-type="epub"><day>13</day><month>07</month><year>2021</year></pub-date><volume>17</volume><issue>3</issue><fpage>492</fpage><lpage>497</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Proshlyakov A.Y., Chomakhidze P.S., Novikova N.A., 2021</copyright-statement><copyright-year>2021</copyright-year><copyright-holder xml:lang="ru">Прошляков А.Ю., Чомахидзе П.Ш., Новикова Н.А.</copyright-holder><copyright-holder xml:lang="en">Proshlyakov A.Y., Chomakhidze P.S., Novikova N.A.</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.rpcardio.online/jour/article/view/2514">https://www.rpcardio.online/jour/article/view/2514</self-uri><abstract><p>Congenital long QT syndrome is a pathology that requires special attention and knowledge about the safety and effectiveness of various medications. Prolongation of the QT interval due to congenital or acquired causes is an important factor in the development of an unfavorable life forecast with the formation of an elongated QT syndrome. With an unfavorable course, patients suffer from loss of consciousness, episodes of tachycardia. Often, stable polymorphic ventricular tachycardia develops. The risk of sudden cardiac death in this pathology can vary from 0.33% to 5%. In people who have suffered an episode of cardiac arrest, and do not have a permanent prescribed antiarrhythmic therapy, the mortality rate reaches 50% within 15 years. Preventive administration of antiarrhythmic drugs is not always effective. A positive result of treatment depends on the severity of long QT syndrome and its genotype. Beta-blockers are often prescribed to patients of different ages with various cardiac pathologies, including for the prevention of arrhythmia in long QT syndrome. Beta-blockers differ in various pharmacokinetic and pharmacodynamic parameters (lipophilicity/hydrophilicity, selectivity, presence/absence of internal sympathomimetic activity), which, along with the variant of the disease genotype, can affect their effectiveness and safety in the considered pathology. This review article presents the results of major studies on the safety and effectiveness of different groups of beta blockers in various variants of long QT syndrome. The preferred beta-blockers for various genotypes of the syndrome were determined, and a comparative characteristic of beta-blockers for their safety and preventive effectiveness was given.</p></abstract><trans-abstract xml:lang="ru"><p>Врожденный синдром удлиненного интервала QT является патологией, требующей особого внимания и знаний по безопасности и эффективности применения различных препаратов. Удлинение интервала QT в силу врожденных или приобретенных причин является важным фактором в развитии неблагоприятного жизненного прогноза с формированием данного синдрома. При неблагоприятном течении пациенты страдают от потерь сознания, эпизодов тахикардии, нередко развивается устойчивая полиморфная желудочковая тахикардия. Риск внезапной сердечной смерти при данной патологии может варьировать от 0,33% до 5%. У лиц, перенесших эпизод остановки сердца, и не имеющих постоянной назначенной антиаритмической терапии, летальность достигает 50% в течение 15 лет. Профилактическое назначение антиаритмических препаратов эффективно не всегда. Положительный результат лечения зависит от тяжести синдрома удлиненного интервала QT и от его молекулярно-генетического варианта. Бета-адреноблокаторы часто назначают пациентам разного возраста при различной кардиальной патологии, в том числе, для профилактики аритмии при синдроме удлиненного интервала QT. Бета-адреноблокаторы отличаются по различным фармакокинетическим и фармакодинамическим параметрам (липофильность/ гидрофильность, селективность, длительность действия, на-личие/отсутствие внутренней симпатомиметической активности), что, наряду с молекулярно-генетическим вариантом заболевания, может повлиять на их эффективность и безопасность при рассматриваемой патологии. В данной обзорной статье изложены результаты основных крупных исследований по безопасности и эффективности применения разных подгрупп бета- адреноблокаторов при различных вариантах синдрома удлиненного интервала QT. Определены предпочтительные бета-адреноблокаторы при различных молекулярно-генетических вариантах синдрома, дана сравнительная характеристика этих лекарственных средств по их безопасности и профилактической эффективности.</p></trans-abstract><kwd-group xml:lang="ru"><kwd>бета-адреноблокаторы</kwd><kwd>удлиненный интервал QT</kwd><kwd>пропранолол</kwd><kwd>метопролол</kwd><kwd>атенолол</kwd><kwd>надолол</kwd><kwd>бисопролол</kwd></kwd-group><kwd-group xml:lang="en"><kwd>beta-blockers</kwd><kwd>prolonged QT interval</kwd><kwd>propranolol</kwd><kwd>metoprolol</kwd><kwd>atenolol</kwd><kwd>nadolol</kwd><kwd>bisoprolol</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">Rautaharju PM, Surawicz B, Gettes LS. 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DOI:10.1016/j.hrthm.2016.09.012.</mixed-citation></citation-alternatives></ref></ref-list><fn-group><fn fn-type="conflict"><p>The authors declare that there are no conflicts of interest present.</p></fn></fn-group></back></article>
