Пролапс митрального клапана: современные представления и нерешенные вопросы (обзор)
Пролапс митрального клапана (ПМК) — наиболее распространенная клапанная аномалия. Многие позиции, касающиеся его диагностики, эпидемиологии, прогноза и генетических основ, в последнее время были уточнены или пересмотрены.
Наиболее принципиальные изменения, касающиеся диагностики ПМК, связаны с установлением трехмерной — седловидной — формы митрального клапанного кольца, что сделало обязательным при проведении ультразвукового исследования оценку состояния клапана из парастернальной продольной позиции. Внедрение стандартных диагностических критериев, основанных на двухмерной эхокардиографии, и обнародование результатов Фремингемского исследования позволили преодолеть противоречия во взглядах на распространенность этой патологии, которая оказалась значительно ниже, чем считалось ранее. Установлены возрастные, гендерные и этнические закономерности встречаемости ПМК. Переоценке подверглись представления не только о частоте развития митрального пролапса, но и о тяжести его последствий. Если ранее ПМК считался заболеванием с регулярными серьезными осложнениями, то результаты проведенных эпидемиологических исследований дали основание рассматривать его как доброкачественную патологию с низкой вероятностью неблагоприятных последствий. Одновременно были выделены факторы неблагоприятного прогноза, главным из которых признана митральная регургитация.
Существенно обогатили представления о ПМК и улучшили его диагностику результаты молекулярно-генетических исследований. В настоящее время эта патология сердца представляется следствием множественных генетических нарушений, что обусловлено идентификацией нескольких генов, имеющих отношение к возникновению синдромного пролапса, и трех локусов — для несиндромного. Создание масштабных регистров больных ПМК и проведение полногеномных исследований позволит идентифицировать новые гены, имеющие отношение к возникновению митрального пролабирования, и обеспечить скрининг бессимптомных пациентов. Ведущую роль в разнообразных механизмах патогенеза ПМК играет нарушение регуляции трансформирующего фактора роста бета (TGF-β), понимание патогенетической роли которого открывает новые перспективы консервативного лечения этой патологии с применением антител, нейтрализующих TGF-β, и блокаторов рецепторов ангиотензина II. Подобные медицинские подходы могут быть перспективны на ранней стадии недиагностированных фенотипов ПМК, а также служить альтернативой хирургического лечения клинических осложнений у пациентов с верифицированным диагнозом.
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