Cardiomyopathies have been indicated to be one of the leading causes of heart failure. Though it was indicated that genetic defects, viral infection and trace element deficiency were among the causes of cardiomyopathy, the etiology has remained to be fully elucidated. Cardiomyocytes require large amounts of energy to maintain their normal biological functions. Adenosine triphosphate-sensitive potassium channels (KATP), composed of inward-rectifier potassium ion channel and sulfonylurea receptor subunits, are present on the cell surface and mitochondrial membrane of cardiac muscle cells. As metabolic sensors sensitive to changes in intracellular energy levels, KATP adapt electrical activities to metabolic challenges, maintaining normal biological functions of myocytes. It is implied that malfunctions, mutations and altered expression of KATP are associated with the pathogenesis of conditions including c hypertrophy, diabetes as well as dilated, ischemic and endemic cardiomyopathy. However, the current knowledge is only the tip of the iceberg and the roles of KATP in cardiomyopathies largely remain to be elucidated in future studies.