Некомпактность левого желудочка: самостоятельная кардиомиопатия или сопутствующий анатомический синдром?

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.

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