LMNA–ассоциированная дилатационная кардиомиопатия

Ламин-ассоциированная дилатационная кардиомиопатия – это генетические заболевания, являющиеся причиной быстропрогрессирующей сердечной недостаточности (СН), различных аритмий и нарушений проводимости и высокого риска внезапной сердечной смерти (ВСС). Представлено клиническое наблюдение пациента с кардиоламинопатией, обусловленной мутациями в гене LMNA, ранними проявлениями которой были быстропрогрессирующая СН, систолическая дисфункция желудочков сердца, аномалия клапанного аппарата, наджелудочковые и желудочковые нарушения ритма и проводимости. Показана эволюция клинических проявлений за период наблюдения, и обсуждены вопросы стратификации риска внезапной сердечной смерти и стратегия ее профилактики при данной патологии.

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