2. Современная диагностика и лечение гипертрофической кардиомиопатии

Авторы

  • им. А.Н. Сызганова ННЦХ Сызганов А.Н. атындағы ҰҒХО АҚ, Алматы қ., Қазақстан
  • И.Е. Сагатов Сызганов А.Н. атындағы ҰҒХО АҚ, Алматы қ., Қазақстан
  • С.О. Шабельянов Сызганов А.Н. атындағы ҰҒХО АҚ, Алматы қ., Қазақстан
  • А.Б. Ерсейт Сызганов А.Н. атындағы ҰҒХО АҚ, Алматы қ., Қазақстан

Загрузки

Аннотация

Введение. Гипертрофическая кардиомиопатия–структурнаяи функциональнаяпатологияхарактеризующа-ясязначительно утолщённой стенкой миокарда желудочков. Распространённость ГК 1на500. До 60% случаев у взрослых является аутосомно–доминантнымзаболеванием при котором имеется мутации в генах кодирующих белок саркомер. История болезни и клиническая картина. Клиническая картина разнообразна и зависит от пенетрантности генов вовлечённых в данную патологию. Большинство пациенты не предъявляют никаких жалоб. Самыми частымисимптомами являются одышка, затрудненное дыхание, утомляемость, припухлость в области лодыжек, ступней, живота и вен шеи. Часто сопровождается обструкцией левого желудочка и проявляется такими симптомами как одышка, загрудинные боли и потеря сознания. Диагностика. В диагностике ГК важную роль играют ЭКГ, ЭХО-КГ, МРТ и МСКТ. У взрослых гипертрофическая кардиомиопатия диагностируется при увеличе-нии стенки левого желудочка больше 15 мм в одном или нескольких сегментах левого желудочка, которые уста-навливаются при помощи любого общепринятого метода диагностики. Лечение. В медикаментозном лечении ГК основную роль занимают Б-блокаторы и блокаторы кальциевых каналов. При неэффективности медикаментозной терапии применяют хирургическую тактику. Существуют два основных инвазивных метода лечения ГК: миоэкто-мия и алкогольная абляция. Миоэктомия применяется с начала 1960-х годов и является открытой операцией, что сопровождается более частыми осложнениями. На данный момент миоэктомия является «золотым стандартом» которая даёт сравнительно лучшие инструментальные и симптоматические показатели в сравнении с алкогольной абляцией.

Заключение. Основополагающим методом в диагностике ГК является ЭХО-КГ. Для дифференциаль-ной диагностики и стратификации риска пациента лучше подходит МРТ. В настоящее времяне существует ран-домизированного клинического исследования для сравнения миоэктомии и алкогольной абляции, но несколько мета-анализов показали, что оба метода уменьшают симптомы и имеют одинаковую смертность. Выбор тактики лечения должна решать многопрофильная команда.

Ключевые слова

гипертрофическая кардиоми-опатия, ЭХО-КГ, МРТ, мутация

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Опубликован

2020-04-01

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№ 1 (2020)

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