Случай редкой формы аритмогенной кардиомиопатии у ребенка 5 лет

   Аритмогенная кардиомиопатия — генетически детерминированное заболевание сердечной мышцы, характеризующееся фиброзно-жировым замещением миокарда желудочков, которое предрасполагает к желудочковым нарушениям ритма и высокому риску внезапной сердечной смерти (ВСС). Изначально считалось, что для данного заболевания характерно исключительное или преимущественное поражение правого желудочка. Однако фиброзно-жировое замещение может также распространяться и на левый желудочек или поражать его преимущественно без вовлечения в процесс правых камер сердца. В данной статье представлен редкий клинический случай — ребенок с синдромом Карвахаль с классической триадой признаков (леводоминантная форма аритмогенной кардиомиопатии, кератодермия и шерстисто-курчавые волосы).

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