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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">russjcardiol</journal-id><journal-title-group><journal-title xml:lang="ru">Российский кардиологический журнал</journal-title><trans-title-group xml:lang="en"><trans-title>Russian Journal of Cardiology</trans-title></trans-title-group></journal-title-group><issn pub-type="ppub">1560-4071</issn><issn pub-type="epub">2618-7620</issn><publisher><publisher-name>«SILICEA-POLIGRAF» LLC</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="doi">10.15829/1560-4071-2014-5-49-54</article-id><article-id custom-type="elpub" pub-id-type="custom">russjcardiol-69</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>МИОКАРДИТЫ, КЛАПАННЫЕ И НЕКОРОНАРОГЕННЫЕ ЗАБОЛЕВАНИЯ</subject></subj-group><subj-group subj-group-type="section-heading" xml:lang="en"><subject>MYOCARDITISES, VALVULAR AND NONCORONAROGENIC DISEASES</subject></subj-group></article-categories><title-group><article-title>ДВУХСТВОРЧАТЫЙ АОРТАЛЬНЫЙ КЛАПАН (ЭВОЛЮЦИЯ ВЗГЛЯДОВ НА ОСОБЫЙ ТИП ВАЛЬВУЛОПАТИИ)</article-title><trans-title-group xml:lang="en"><trans-title>BICUSPID AORTIC VALVE (A DEVELOPMENT OF INSIGHT INTO VALVULOPATHIES)</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>Дземешкевич</surname><given-names>С. Л.</given-names></name><name name-style="western" xml:lang="en"><surname>Dzemeshkevitch</surname><given-names>S. L.</given-names></name></name-alternatives><bio xml:lang="ru"><p>профессор, д. м.н., руководитель отделения хирургического лечения дисфункций миокарда и сердечной недостаточности, директор ФГБУ РНЦХ им. акад. Б. В. Петровского РАМН, руководитель отделения хирургического лечения дисфункций миокарда и сердечной недостаточности</p></bio><email xlink:type="simple">dzem@med.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>Иванов</surname><given-names>В. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Ivanov</surname><given-names>V. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д. м.н., профессор, руководитель отделения хирургии пороков сердца</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Чарчян</surname><given-names>Э. Р.</given-names></name><name name-style="western" xml:lang="en"><surname>Charchian</surname><given-names>E. R.</given-names></name></name-alternatives><bio xml:lang="ru"><p>д. м.н., руководитель отделения хирургии аорты и ее ветвей</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Евсеев</surname><given-names>Е. П.</given-names></name><name name-style="western" xml:lang="en"><surname>Evseev</surname><given-names>E. P.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к. м.н., в. н.с. отделения хирургии пороков сердца</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Фролова</surname><given-names>Ю. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Frolova</surname><given-names>Yu. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к. м.н., в. н.с. отделения хирургического лечения дисфункций миокарда и сердечной недостаточности</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Луговой</surname><given-names>А. Н.</given-names></name><name name-style="western" xml:lang="en"><surname>Lugovoy</surname><given-names>A. N.</given-names></name></name-alternatives><bio xml:lang="ru"><p>аспирант отделения хирургического лечения дисфункций миокарда и сердечной недостаточности</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Федулова</surname><given-names>С. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Fedulova</surname><given-names>S. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к. м.н., с. н.с. лаборатории интраоперационной диагностики</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Ховрин</surname><given-names>В. В.</given-names></name><name name-style="western" xml:lang="en"><surname>Khovrin</surname><given-names>V. V.</given-names></name></name-alternatives><bio xml:lang="ru"><p>к. м.н., руководитель отделением рентгенодиагностики и компьютерной томографии</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><name-alternatives><name name-style="eastern" xml:lang="ru"><surname>Букаева</surname><given-names>А. А.</given-names></name><name name-style="western" xml:lang="en"><surname>Bukaeva</surname><given-names>A. A.</given-names></name></name-alternatives><bio xml:lang="ru"><p>лаборант лаборатории медицинской генетики</p></bio><xref ref-type="aff" rid="aff-2"/></contrib><contrib contrib-type="author" corresp="yes"><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. м.н., профессор, руководитель лабораторией медицинской генетики</p></bio><xref ref-type="aff" rid="aff-1"/></contrib></contrib-group><aff-alternatives id="aff-1"><aff xml:lang="ru"><institution>ФГБУ Российский научный центр хирургии им. академика Б. В. Петровского РАМН, Москва, Россия</institution><country>Россия</country></aff><aff xml:lang="en"><institution>FSBI Petrovsky Russian Scientific Centre for Surgery of the Academy of Medical Sciences, Moscow, Russia</institution><country>Russian Federation</country></aff></aff-alternatives><aff-alternatives id="aff-2"><aff xml:lang="ru"><institution>ФГБУ Российский научный центр хирургии им. академика Б. В. Петровского РАМН, Москва, Россия</institution><country>Россия</country></aff><aff xml:lang="en"><institution>FSBI Petrovsky Russian Scientific Centre for Surgery of the Academy of Medical Sciences, Moscow, Russia.</institution><country>Russian Federation</country></aff></aff-alternatives><pub-date pub-type="collection"><year>2014</year></pub-date><pub-date pub-type="epub"><day>28</day><month>05</month><year>2014</year></pub-date><volume>0</volume><issue>5</issue><fpage>49</fpage><lpage>54</lpage><permissions><copyright-statement>Copyright &amp;#x00A9; Дземешкевич С.Л., Иванов В.А., Чарчян Э.Р., Евсеев Е.П., Фролова Ю.В., Луговой А.Н., Федулова С.В., Ховрин В.В., Букаева А.А., Заклязьминская Е.В., 2014</copyright-statement><copyright-year>2014</copyright-year><copyright-holder xml:lang="ru">Дземешкевич С.Л., Иванов В.А., Чарчян Э.Р., Евсеев Е.П., Фролова Ю.В., Луговой А.Н., Федулова С.В., Ховрин В.В., Букаева А.А., Заклязьминская Е.В.</copyright-holder><copyright-holder xml:lang="en">Dzemeshkevitch S.L., Ivanov V.A., Charchian E.R., Evseev E.P., Frolova Y.V., Lugovoy A.N., Fedulova S.V., Khovrin V.V., Bukaeva A.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://russjcardiol.elpub.ru/jour/article/view/69">https://russjcardiol.elpub.ru/jour/article/view/69</self-uri><abstract><sec><title>Материал и методы</title><p>Материал и методы. В исследование включены 207 пациентов, которые в течение пяти лет были оперированы в связи с прогрессированием врожденного аортального порока. Возраст оперированных варьировал от 17 до 75 лет (средний возраст — 49,6±0,9 лет), соотношение мужчин и женщин — 3:1. Сочетание с ревматизмом выявлено у 33 (15,9%) пациентов. Перенесенный инфекционный эндокардит отмечен у 45 (21,7%) пациентов. Пациентов с доминирующим стенозом было 111 (53,6%). Расширение восходящей аорты выявлено у 82 пациентов (39,6%).</p></sec><sec><title>Результаты</title><p>Результаты. У большинства пациентов были использованы механические протезы и только у 12 биопротезы; 13 пациентам сделали пластику клапана либо вмешательства на створках не потребовалось. Треть пациентов (31,9%) нуждались в протезировании восходящей аорты. Сочетанные дополнительные процедуры (пластика или протезирование аортального клапана, пластика трикуспидального клапана, пластика левого предсердия, ушивание септальных дефектов, аортокоронарное шунтирование) были нередкими — 57. Госпитальная летальность составила 2,9%.</p></sec><sec><title>Заключение</title><p>Заключение. Бикуспидальный аортальный клапан является особым видом вальвулопатии, нередко сочетающейся с аортопатией. Генез патологии носит, вероятнее всего, генетический характер. Клинические проявления порока развиваются со временем и могут быть ускорены при развитии ревматизма или инфекционного эндокардита. Хирургическое лечение эффективно.</p></sec><sec><title> </title><p> </p></sec></abstract><trans-abstract xml:lang="en"><sec><title>Aim</title><p>Aim. To study different ways of development and combinational abilities of pathological changes common for bicuspid aortic valvulopathy. Material and methods. Totally 207 patients included, who had during previous 5 years been operated for progression of inherited aortic valve defect. The age varied from 17 to 75 years (median 49,6±0,9 y.), men to women — 3:1. Comorbidities included rheumatic fever in 33 (15,9%) patients and infectious endocarditis in the past in 45 (21,7%). In 111 (53,6%) patients aortic stenosis dominated and distension of ascending aorta — in 82 (39,6%) patients.</p></sec><sec><title>Results</title><p>Results. For most of patients mechanical prostheses were used and only for 12 — biological; 13 patients underwent valvulopastic or no any operation. One third of patients (31,9%) required a prosthesis of ascending aorta. Combinations of additional procedures (as aortic valve plastic or prosthesis, tricuspid plastic, left atrium plastic, septal defects sewing, coronary bypass grafting) were not unusual — in 57. In-hospital mortality reached 2,9%.</p></sec><sec><title>Conclusion</title><p>Conclusion. Bicuspid aortic valve is a specific kind of valvulopathy, commonly combined with aortopathy. The development of this pathology is probably inherited. Clinical picture of the defect develops during time and its exacerbation might be accelerated by rheumathic fever and infectious endocarditis. Surgical treatment is effective.</p></sec></trans-abstract><kwd-group xml:lang="ru"><kwd>Бикуспидальный аортальный клапан</kwd><kwd>аортопатия</kwd><kwd>протезирование клапана</kwd><kwd>генетика соединительно-тканных дисплазий</kwd></kwd-group><kwd-group xml:lang="en"><kwd>bicuspid aortic valve</kwd><kwd>aortopathy</kwd><kwd>valve prosthesis</kwd><kwd>the genetics of connective tissue dysplasia</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">Dzemeshkevich S. L., Stevenson SL, Alexi-Meskhishvili VV. Disease aortic valve (function, diagnostics, treatment). M., GEOTAR-MED, 2004, 325 C. Russian (Дземешкевич С. Л., Стивенсон Л. У., Алекси-Месхишвили В. В. Болезни аортального клапана (функция, диагностика, лечение). М., ГЭОТАР-МЕД, 2004, 325 с).</mixed-citation><mixed-citation xml:lang="en">Dzemeshkevich S. L., Stevenson SL, Alexi-Meskhishvili VV. Disease aortic valve (function, diagnostics, treatment). M., GEOTAR-MED, 2004, 325 C. Russian (Дземешкевич С. Л., Стивенсон Л. У., Алекси-Месхишвили В. В. Болезни аортального клапана (функция, диагностика, лечение). М., ГЭОТАР-МЕД, 2004, 325 с).</mixed-citation></citation-alternatives></ref><ref id="cit2"><label>2</label><citation-alternatives><mixed-citation xml:lang="ru">Siu S, Silversides C. Bicuspid Aortic Valve Disease. J Am Coll Cardiol 2010; 55: 2789-800.</mixed-citation><mixed-citation xml:lang="en">Siu S, Silversides C. Bicuspid Aortic Valve Disease. J Am Coll Cardiol 2010; 55: 2789-800.</mixed-citation></citation-alternatives></ref><ref id="cit3"><label>3</label><citation-alternatives><mixed-citation xml:lang="ru">Mordi J, Tzemos N. Bicuspid Aorte Valve Disease: a comprehensive review. Cardiology Research and Practice. Hindawi Publishing Corporation, volume 2012, p. 2-5.</mixed-citation><mixed-citation xml:lang="en">Mordi J, Tzemos N. Bicuspid Aorte Valve Disease: a comprehensive review. Cardiology Research and Practice. Hindawi Publishing Corporation, volume 2012, p. 2-5.</mixed-citation></citation-alternatives></ref><ref id="cit4"><label>4</label><citation-alternatives><mixed-citation xml:lang="ru">Padang R, Bagnall R, Semsarian Ch. Genetic Basis of Familial Valvular Heart disease. Circ Cardiovasc Genet, 2012, 5: 569-80.</mixed-citation><mixed-citation xml:lang="en">Padang R, Bagnall R, Semsarian Ch. Genetic Basis of Familial Valvular Heart disease. Circ Cardiovasc Genet, 2012, 5: 569-80.</mixed-citation></citation-alternatives></ref><ref id="cit5"><label>5</label><citation-alternatives><mixed-citation xml:lang="ru">Padang R, Bannon P, Jeremy R, et al. The genetic and molecular basis of bicuspid aortic valve associated thoracic aortopathy: a link to phenotype heterogenecity. Ann Cardiothorac Surg, 2013; 2 (1): 83-91.</mixed-citation><mixed-citation xml:lang="en">Padang R, Bannon P, Jeremy R, et al. The genetic and molecular basis of bicuspid aortic valve associated thoracic aortopathy: a link to phenotype heterogenecity. Ann Cardiothorac Surg, 2013; 2 (1): 83-91.</mixed-citation></citation-alternatives></ref><ref id="cit6"><label>6</label><citation-alternatives><mixed-citation xml:lang="ru">Lokshin PS. Artificial circulation. In the book: National guide "Anesthesiology", Ed. But, GEOTAR, M,2011, s.626-48. Russian (Локшин Л. С. Искусственное кровообращение. В кн.: Национальное руководство "Анестезиология", ред. А.А. Бунятян, ГЭОТАР, М.,2011, с.626-48).</mixed-citation><mixed-citation xml:lang="en">Lokshin PS. Artificial circulation. In the book: National guide "Anesthesiology", Ed. But, GEOTAR, M,2011, s.626-48. Russian (Локшин Л. С. Искусственное кровообращение. В кн.: Национальное руководство "Анестезиология", ред. А.А. Бунятян, ГЭОТАР, М.,2011, с.626-48).</mixed-citation></citation-alternatives></ref><ref id="cit7"><label>7</label><citation-alternatives><mixed-citation xml:lang="ru">Trekova NA. Anesthetic management operations on the heart and the aorta. Anesthesiology and reanimatology) 2013, 2: 6-10. Russian (Трекова Н. А. Анестезиологическое обеспечение операций на сердце и аорте. Анестезиология и реаниматология 2013, 2: 6-10).</mixed-citation><mixed-citation xml:lang="en">Trekova NA. Anesthetic management operations on the heart and the aorta. Anesthesiology and reanimatology) 2013, 2: 6-10. Russian (Трекова Н. А. Анестезиологическое обеспечение операций на сердце и аорте. Анестезиология и реаниматология 2013, 2: 6-10).</mixed-citation></citation-alternatives></ref><ref id="cit8"><label>8</label><citation-alternatives><mixed-citation xml:lang="ru">Dzemeshkevich SL, Dementieva II, Zhidkov IL, et al. Long-term conservation of the heart in the solution of the Console and Custodial (experimental study). Transplantul. and arts. bodies, 1996; 3-4: 67-74. Russian (Дземешкевич С. Л., Дементьева И. И., Жидков И. Л. и др. Длительная консервация сердца в растворе Консол и Кустодиол (экспериментальное исследование). Трансплантол. и искусств. органы, 1996; 3-4: 67-74).</mixed-citation><mixed-citation xml:lang="en">Dzemeshkevich SL, Dementieva II, Zhidkov IL, et al. Long-term conservation of the heart in the solution of the Console and Custodial (experimental study). Transplantul. and arts. bodies, 1996; 3-4: 67-74. Russian (Дземешкевич С. Л., Дементьева И. И., Жидков И. Л. и др. Длительная консервация сердца в растворе Консол и Кустодиол (экспериментальное исследование). Трансплантол. и искусств. органы, 1996; 3-4: 67-74).</mixed-citation></citation-alternatives></ref><ref id="cit9"><label>9</label><citation-alternatives><mixed-citation xml:lang="ru">Warnes CA, Williams RC, Bashore TM, et al. ACC/AHA 2008 Guidelines for the management of adult with congenital heart disease. J Am Coll Cardiol 2008; 52: 1-121.</mixed-citation><mixed-citation xml:lang="en">Warnes CA, Williams RC, Bashore TM, et al. ACC/AHA 2008 Guidelines for the management of adult with congenital heart disease. J Am Coll Cardiol 2008; 52: 1-121.</mixed-citation></citation-alternatives></ref><ref id="cit10"><label>10</label><citation-alternatives><mixed-citation xml:lang="ru">Vahanian A, Alfieri O, Andreotti T, et al. Guidelines on the management of valvular heart disease (version 2012) of the ESC and EACTS. European Heart Journal (2012); 33: 2451-96.</mixed-citation><mixed-citation xml:lang="en">Vahanian A, Alfieri O, Andreotti T, et al. Guidelines on the management of valvular heart disease (version 2012) of the ESC and EACTS. European Heart Journal (2012); 33: 2451-96.</mixed-citation></citation-alternatives></ref><ref id="cit11"><label>11</label><citation-alternatives><mixed-citation xml:lang="ru">Krylova NS, Poteshkina N. G. Gipertrophy myopathy in the elderly. Heart failure, 2011; 12, 5: 309-11. Russian (Крылова Н. С., Потешкина Н. Г. Гипетрофическая кардиомиопатия у лиц пожилого возраста. Сердечная недостаточность, 2011; 12, 5: 309-11).</mixed-citation><mixed-citation xml:lang="en">Krylova NS, Poteshkina N. G. Gipertrophy myopathy in the elderly. Heart failure, 2011; 12, 5: 309-11. Russian (Крылова Н. С., Потешкина Н. Г. Гипетрофическая кардиомиопатия у лиц пожилого возраста. Сердечная недостаточность, 2011; 12, 5: 309-11).</mixed-citation></citation-alternatives></ref><ref id="cit12"><label>12</label><citation-alternatives><mixed-citation xml:lang="ru">Maron B, Casey S, Hauser R, et al. Clinical course of hypertrophic cardiomyopathy with survival to advanced age. J Am Coll Cardiol, 2003; 42 (5): 882-8.</mixed-citation><mixed-citation xml:lang="en">Maron B, Casey S, Hauser R, et al. Clinical course of hypertrophic cardiomyopathy with survival to advanced age. J Am Coll Cardiol, 2003; 42 (5): 882-8.</mixed-citation></citation-alternatives></ref><ref id="cit13"><label>13</label><citation-alternatives><mixed-citation xml:lang="ru">Erscan T., Ekisi F., Atalay S. et al. The prevalence of bicuspid aortic valve in newborns by echocardiography screening. American Heart Journal, 2005; 150, 3: 513-5.</mixed-citation><mixed-citation xml:lang="en">Erscan T., Ekisi F., Atalay S. et al. The prevalence of bicuspid aortic valve in newborns by echocardiography screening. American Heart Journal, 2005; 150, 3: 513-5.</mixed-citation></citation-alternatives></ref><ref id="cit14"><label>14</label><citation-alternatives><mixed-citation xml:lang="ru">Stewart S., Ahmed R., Travill C. et al. Coarctation of the aorta life and health 20-44 years after surgery repair. British Heart Journal, 1993; 69, 1: 65-70.</mixed-citation><mixed-citation xml:lang="en">Stewart S., Ahmed R., Travill C. et al. Coarctation of the aorta life and health 20-44 years after surgery repair. British Heart Journal, 1993; 69, 1: 65-70.</mixed-citation></citation-alternatives></ref><ref id="cit15"><label>15</label><citation-alternatives><mixed-citation xml:lang="ru">Fedak P, Verma S, David T, et al. Clinical and pathophysiological implications of a bicuspid aortic valve. Circulation, 2002; 106, 8: 900-4.</mixed-citation><mixed-citation xml:lang="en">Fedak P, Verma S, David T, et al. Clinical and pathophysiological implications of a bicuspid aortic valve. Circulation, 2002; 106, 8: 900-4.</mixed-citation></citation-alternatives></ref><ref id="cit16"><label>16</label><citation-alternatives><mixed-citation xml:lang="ru">Calloway T, Martin J, Zhang X, et al. Risk factors for aortic valve disease in bicuspid aortic valve: a family-based study. Am J Med Genet A, 2011; 155, 5: 1015-20.</mixed-citation><mixed-citation xml:lang="en">Calloway T, Martin J, Zhang X, et al. Risk factors for aortic valve disease in bicuspid aortic valve: a family-based study. Am J Med Genet A, 2011; 155, 5: 1015-20.</mixed-citation></citation-alternatives></ref><ref id="cit17"><label>17</label><citation-alternatives><mixed-citation xml:lang="ru">Dzemeshkevich A. S., Ruskin CENTURIES, Malikova MS et al. Dysplasia mitral valve prolapse in adults: the choice of surgical technique. Surgery, 2013; 2: 40-5. Russian (Дземешкевич А. С., Раскин В. В., Маликова М. С. и др. Дисплазии митрального клапана у взрослых: выбор хирургической методики. Хирургия, 2013; 2: 40-5).</mixed-citation><mixed-citation xml:lang="en">Dzemeshkevich A. S., Ruskin CENTURIES, Malikova MS et al. Dysplasia mitral valve prolapse in adults: the choice of surgical technique. Surgery, 2013; 2: 40-5. Russian (Дземешкевич А. С., Раскин В. В., Маликова М. С. и др. Дисплазии митрального клапана у взрослых: выбор хирургической методики. Хирургия, 2013; 2: 40-5).</mixed-citation></citation-alternatives></ref><ref id="cit18"><label>18</label><citation-alternatives><mixed-citation xml:lang="ru">Hahn R, Roman M, Mogtader A, et al. Association of aortic dilatation with regurgitant, stenotic and functionally normal bicuspid aortic valves. J Am Coll Cardiol 1992; 19: 283-8.</mixed-citation><mixed-citation xml:lang="en">Hahn R, Roman M, Mogtader A, et al. Association of aortic dilatation with regurgitant, stenotic and functionally normal bicuspid aortic valves. J Am Coll Cardiol 1992; 19: 283-8.</mixed-citation></citation-alternatives></ref><ref id="cit19"><label>19</label><citation-alternatives><mixed-citation xml:lang="ru">Bonderman D, Charchbaghi-Schnell E, Maurer G, et al. Mechanisms underlying aortic dilatation in congenital aortic valve malformation. Circulation 1999; 99: 2138-43.</mixed-citation><mixed-citation xml:lang="en">Bonderman D, Charchbaghi-Schnell E, Maurer G, et al. Mechanisms underlying aortic dilatation in congenital aortic valve malformation. Circulation 1999; 99: 2138-43.</mixed-citation></citation-alternatives></ref><ref id="cit20"><label>20</label><citation-alternatives><mixed-citation xml:lang="ru">Fedak P, de Sa M, Verma S, et al. Vascular matrix remodeling in patients with bicuspid aortic valve malformations: implications for aortic dilatation. J Thorac Catdiovasc Surg 2003; 126: 797-806.</mixed-citation><mixed-citation xml:lang="en">Fedak P, de Sa M, Verma S, et al. Vascular matrix remodeling in patients with bicuspid aortic valve malformations: implications for aortic dilatation. J Thorac Catdiovasc Surg 2003; 126: 797-806.</mixed-citation></citation-alternatives></ref><ref id="cit21"><label>21</label><citation-alternatives><mixed-citation xml:lang="ru">Boyum J, Fellinger E, Schmoker J, et al. Matrix metalloproteinase activity in thoracic aortic aneurisms associated with bicuspid and tricuspid aortic valves. J Thorac Cardiovasc.Surg, 2004; 127: 686-91.</mixed-citation><mixed-citation xml:lang="en">Boyum J, Fellinger E, Schmoker J, et al. Matrix metalloproteinase activity in thoracic aortic aneurisms associated with bicuspid and tricuspid aortic valves. J Thorac Cardiovasc.Surg, 2004; 127: 686-91.</mixed-citation></citation-alternatives></ref><ref id="cit22"><label>22</label><citation-alternatives><mixed-citation xml:lang="ru">Loscalzo M, Goh D, Loeys B, et al. Familial thoracic aortic dilatation and bicomissural aortic valve: a prospective analysis of natural history and inheritance. Am J Med Genet A, 2007; 143A: 1960-7.</mixed-citation><mixed-citation xml:lang="en">Loscalzo M, Goh D, Loeys B, et al. Familial thoracic aortic dilatation and bicomissural aortic valve: a prospective analysis of natural history and inheritance. Am J Med Genet A, 2007; 143A: 1960-7.</mixed-citation></citation-alternatives></ref><ref id="cit23"><label>23</label><citation-alternatives><mixed-citation xml:lang="ru">OMIM (On-line Mendelian Inheritance in Man) www.ncbi.nlm.nih.gov/omim</mixed-citation><mixed-citation xml:lang="en">OMIM (On-line Mendelian Inheritance in Man) www.ncbi.nlm.nih.gov/omim</mixed-citation></citation-alternatives></ref><ref id="cit24"><label>24</label><citation-alternatives><mixed-citation xml:lang="ru">Pepe G, Nistri S, Giusti B, et al. Identification of fibrillin 1 gene mutations in patients with bicuspid aortic valve (BAV) without Marfan syndrome. BMC Medical Genetics 2014, 15: 23.</mixed-citation><mixed-citation xml:lang="en">Pepe G, Nistri S, Giusti B, et al. Identification of fibrillin 1 gene mutations in patients with bicuspid aortic valve (BAV) without Marfan syndrome. BMC Medical Genetics 2014, 15: 23.</mixed-citation></citation-alternatives></ref><ref id="cit25"><label>25</label><citation-alternatives><mixed-citation xml:lang="ru">Groenink M, den Hartog AW, Franken R, et al. Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial. Eur Heart J. 2013 Dec; 34 (45): 3491-500.</mixed-citation><mixed-citation xml:lang="en">Groenink M, den Hartog AW, Franken R, et al. Losartan reduces aortic dilatation rate in adults with Marfan syndrome: a randomized controlled trial. Eur Heart J. 2013 Dec; 34 (45): 3491-500.</mixed-citation></citation-alternatives></ref><ref id="cit26"><label>26</label><citation-alternatives><mixed-citation xml:lang="ru">Mullen M, Flather M, Jin X, et al. A prospective, randomized, placebo-controlled, double- blind, multicenter study of the effects of irbesartan on aortic dilatation in Marfan syndrome (AIMS trial): study protocol. Mullen et al. Trials 2013, 14: 408.</mixed-citation><mixed-citation xml:lang="en">Mullen M, Flather M, Jin X, et al. A prospective, randomized, placebo-controlled, double- blind, multicenter study of the effects of irbesartan on aortic dilatation in Marfan syndrome (AIMS trial): study protocol. Mullen et al. Trials 2013, 14: 408.</mixed-citation></citation-alternatives></ref><ref id="cit27"><label>27</label><citation-alternatives><mixed-citation xml:lang="ru">Garg V, Muth AN, Ransom JF, et al. Mutations in NOTCH1 cause aortic valve disease. Nature 2005; 437: 270-4.</mixed-citation><mixed-citation xml:lang="en">Garg V, Muth AN, Ransom JF, et al. Mutations in NOTCH1 cause aortic valve disease. Nature 2005; 437: 270-4.</mixed-citation></citation-alternatives></ref><ref id="cit28"><label>28</label><citation-alternatives><mixed-citation xml:lang="ru">Tan HL, Glen E, Topf A, et al. Nonsynonymous variants in the SMAD6 gene predispose to congenital cardiovascular malformation. Hum. Mutat. 2012; 33: 720-7.</mixed-citation><mixed-citation xml:lang="en">Tan HL, Glen E, Topf A, et al. Nonsynonymous variants in the SMAD6 gene predispose to congenital cardiovascular malformation. Hum. Mutat. 2012; 33: 720-7.</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>
