ГИПЕРТРОФИЧЕСКАЯ КАРДИОМИОПАТИЯ: ГЕНЕТИЧЕСКИЕ ИЗМЕНЕНИЯ, ПАТОГЕНЕЗ И ПАТОФИЗИОЛОГИЯ
https://doi.org/10.15829/1560-4071-2014-5-35-42
Аннотация
Настоящий обзор посвящен описанию генетических изменений, патогенетических механизмов и патофизиологии гипертрофической кардиомиопатии на основе анализа последних опубликованных данных. Приведены современные данные о роли выявленных многочисленных мутаций структурных, сократительных и регуляторных белков саркомера в патогенезе кардиомиопатии. Освещены основные гипотезы патогенетического процесса, особое внимание уделено нарушению регуляции обмена кальция. Подчеркнута важность проведения генетического тестирования у больных гипертрофической кардиомиопатией и их родственников. В обзоре обсуждаются основные патофизиологические характеристики заболевания с позиции их диагностической, клинической и прогностической значимости. Кроме описания патофизиологических особенностей, в частности, обструкции выносящего тракта левого желудочка, диастолической дисфункции, ишемии миокарда и нарушений ритма сердца, подчеркнута взаимосвязь этих нарушений с клинической картиной, а также роль современных методов исследования (позитронно-эмиссионная и компьютерная томография) в ранней диагностике и мониторировании клинического течения заболевания.
Об авторах
Н. Т. Ватутин
Донецкий национальный медицинский университет им. М. Горького, Донецк, Украина
Украина
д. м.н., профессор, заведующий кафедрой внутренней медицины № 1
Украина
д. м.н., профессор, заведующий кафедрой внутренней медицины № 1
Г. Г. Тарадин
Донецкий национальный медицинский университет им. М. Горького, Донецк, Украина
Украина
к. м.н., доцент кафедры внутренней медицины № 1
Украина
к. м.н., доцент кафедры внутренней медицины № 1
М. С. Марон
Tuft University School of Medicine, Hypertrophic Cardiomyopathy Center, Boston, Massachusetts, USA
Соединённые Штаты Америки
Assistant Professor, Director of Hypertrophic Cardiomyopathy Center
Соединённые Штаты Америки
Assistant Professor, Director of Hypertrophic Cardiomyopathy Center
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Рецензия
Для цитирования:
Ватутин Н.Т., Тарадин Г.Г., Марон М.С. ГИПЕРТРОФИЧЕСКАЯ КАРДИОМИОПАТИЯ: ГЕНЕТИЧЕСКИЕ ИЗМЕНЕНИЯ, ПАТОГЕНЕЗ И ПАТОФИЗИОЛОГИЯ. Российский кардиологический журнал. 2014;(5):35-42. https://doi.org/10.15829/1560-4071-2014-5-35-42
For citation:
Vatutin N.T., Taradin G.G., Maron M.S. HYPERTROPHIC CARDIOMYOPATHY: GENETIC ALTERATIONS, PATHOGENESIS AND PATHOPHYSIOLOGY. Russian Journal of Cardiology. 2014;(5):35-42. (In Russ.) https://doi.org/10.15829/1560-4071-2014-5-35-42
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