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Muscular Dystrophies (MDs) are frequently not confined to the skeletal muscles but also involve other organs or tissues. In the last few years, remarkable progress has been made in understanding the close and various relations between skeletal muscle disease and heart muscle disease. Cardiac involvement has been documented in a number of primary MDs20 and is even the dominant feature in some of them. Myocardial disease manifesting predominantly as cardiomyopathy and congestive heart failure is characteristic of dystrophies and X-linked dilated cardiomyopathy, whereas conduction system abnormalities that cause heart block, arrhythmias, and sudden death are more commonly seen in limb-girdle type 1B, myotonic, and Emery-Dreifuss muscular dystrophies. Many patients with MD die because of cardiac complications like sudden cardiac death (SCD) or congestive heart failure (CHF). Cardiomyopathy as a result of particular mutations in different muscle genes will be examined and discussed.