Некомпактность левого желудочка: самостоятельная кардиомиопатия или сопутствующий анатомический синдром?
https://doi.org/10.18087/cardio.2558
Аннотация
Некомпактный миокард (НКМ) ЛЖ характеризуется гипертрабекулярностью (утолщенный некомпактный слой) с глубокими межтрабекулярными карманами, которые сообщаются с полостью желудочка, а также тонким компактным уплотненным слоем. Фенотипы НКМ чрезвычайно вариабельны: НКМ левого или ПЖ, бивентрикулярная форма, НКМ с симптомами СН или аритмии, бессимптомные формы или варианты с тромбоэмболическими эпизодами. У 30–50% пациентов с НКМ при генетическом исследовании выявляют мутации генов, кодирующих саркомерные или цитоскелетные белки. В статье представлен литературный обзор по проблемам диагностики, визуализации, патогенеза, вариабельности клинических проявлений НКМ и его генетической гетерогенности. Клинические наблюдения, демонстрирующие НКМ и как сопутствующий анатомический синдром при моногенной болезни Данона, и как семейную форму кардиомиопатии с дигенным наследованием двух фенотипов (НКМ в сочетании с дилатационной кардиомиопатией), а также уникальный случай перипартальной эволюции приобретенного синдрома НКМ отражают текущую неопределенность в отношении патогенеза и значимости НКМ. Основной вопрос, является ли НКМ самостоятельной формой кардиомиопатии или морфологическим признаком и составным анатомическим синдромом врожденных пороков сердца или других первичных форм кардиомиопатии (дилатационной, гипертрофической, аритмогенной правожелудочковой), остается пока дискутабельным. Достижение профессионального рекомендательного консенсуса по унификации диагностических критериев и риск-стратификации НКМ, улучшение средств визуализации и расширение генетического тестирования помогут значительно расширить наши знания и понимание патогенеза, клинической значимости и прогноза НКМ для оптимизации лечебной стратегии.
Об авторах
Т. Г. ВайханскаяБеларусь
220036, Республика Беларусь, Минск, ул. Р. Люксембург, д. 110
Л. Н. Сивицкая
Беларусь
220072, Республика Беларусь, Минск, ул. Академическая, д. 27
Т. В. Курушко
Беларусь
220036, Республика Беларусь, Минск, ул. Р. Люксембург, д. 110
О. Г. Нижникова
Беларусь
220036, Республика Беларусь, Минск, ул. Р. Люксембург, д. 110
О. Д. Левданский
Беларусь
220072, Республика Беларусь, Минск, ул. Академическая, д. 27
Н. Г. Даниленко
Беларусь
220072, Республика Беларусь, Минск, ул. Академическая, д. 27
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Рецензия
Для цитирования:
Вайханская Т.Г., Сивицкая Л.Н., Курушко Т.В., Нижникова О.Г., Левданский О.Д., Даниленко Н.Г. Некомпактность левого желудочка: самостоятельная кардиомиопатия или сопутствующий анатомический синдром? Кардиология. 2018;58(11S):33-45. https://doi.org/10.18087/cardio.2558
For citation:
Vaikhanskaya T.G., Sivitskaya L.N., Kurushko T.V., Nizhnikava O.G., Levdanskiy O.D., Danilenko N.G. Left ventricular noncompaction: a distinct cardiomyopathy or a composite anatomical syndrome? Kardiologiia. 2018;58(11S):33-45. (In Russ.) https://doi.org/10.18087/cardio.2558