Warmest welcome back to Step 1 by NBME lol

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Congenital cardiomegaly (point of maximal impulse is deviated to the left) and hypotonia are both classic features of Pompe disease.

Although GM1 gangliosidosis can present with hepatomegaly and hypotonia, it is not associated with cardiomegaly.

Due to a quick google search, the only way I could see to differentiate GM1 gangliosidosis and Pompes was that Pompes does NOT have splenomegaly. Otherwise, these diseases share many common features.

trying to use logic for this one: glycogen storage diseases cause hepatomegaly (not splenomegaly) because glycogen is stored in the liver (same goes for von Gierke disease, which is type I glycogen storage disease and is in UWorld)