The course of the disease in PRKAG2 cardiomyopathy in our series seems to be related to the site of mutation in the PRKAG2 gene, with severe complications found in carriers of PRKAG2 Phe293Leu, His530Arg, and Val336Leu variants, and not in carriers of the Arg302Gln PRKAG2 variant. The gene discussed is PRKAG2; the disease is cardiomyopathy.