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What is Which disorder of carbohydrate metabolism disorder typically has cardiac involvement? and explain in detail?
Classic infantile form of Pompe disease presents with Cardiomyopathy and conduction disorders Muscular hypotonia / floppy baby Macroglossia Organomegaly Cardiomyopathy- hyperophic type, severe thickening of the septum and free walls of the left and right hea. Glycogen storage involves cardiac myocytes, special cells of the conduction system (A-V node and His-bundle cells), representing the histological background of classical ECG abnormalities in Pompe disease: pre-excitation patterns (sho PR, delta waves), AV blocks and bundle branch abnormalities.