In human hearts, we observed that VLC dihydroceramides correlated with incidence of cardiac arrhythmias and heart failure and that expression of CERS2, a subtype of CERS known to synthesize VLC dihydroceramides, was positively associated with signaling pathways linked to cardiac arrhythmia and cardiomyopathy. This evidence concerns the gene CERS2 and cardiomyopathy.