Which disorder of carbohydrate metabolism disorder typically has cardiac involvement?
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.
Glycogen Storage Disease Type II (Pompe)
Galactosemia
Glycogen Storage Disease Type I (Von-Gierke)
Hereditary fructose intolerance
Takotsubo Cardiomyopathy and Brugada Syndrome