Сочетание аритмогенной кардиомиопатии правого желудочка с синдромом Лоиса-Дитца: клиническое наблюдение

Аритмогенная кардиомиопатия правого желудочка - наследственная кардиомиопатия, частая причина внезапной сердечной смерти у детей и молодых взрослых. Синдром Лоиса-Дитца - редкое наследственное заболевание соединительной ткани, характеризующееся аневризмой аорты и других крупных артерий, патологической извитостью артерий и гипермобильностью суставов, связанное с патогенными вариантами в генах, кодирующих белковые компоненты TGF-β сигнального пути. Представляем случай сочетания двух вышеупомянутых наследственных заболеваний у пробанда с комплексным и быстро прогрессирующим поражением сердечно-сосудистой системы. 

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