Некомпактный миокард левого желудочка
Аннотация
Некомпактный миокард левого желудочка (НМЛЖ) — редкая форма кардиомиопатии, для которой характерна патологическая гипертрабекулярность миокарда левого желудочка с формированием двух слоев миокарда — нормального и некомпактного. Заболевание является генетически детерминированным и развивается в результате нарушения нормальных процессов эмбриогенеза сердца. Патогенез и клиническая картина складываются из трех ведущих синдромов: сердечной недостаточности, аритмий, тромбоэмболического синдрома. Диагностика основана на данных эхокардиографического исследования, результатах магнитно-резонансной томографии сердца. Болезнь имеет неблагоприятное течение и прогноз, при этом летальность во взрослой популяции составляет около 50 %. Лечение является патогенетическим и заключается в терапии сердечной недостаточности, нарушений ритма сердца и профилактике тромбоэмболических осложнений. Пациентам с терминальной стадией сердечной недостаточности показана трансплантация сердца.Литература
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Голухова Елена Зеликовна,Шомахов Руслан Анатольевич.
