1. Common cardiomyopathies include hypertrophic Cardiomyopathy and dilated Cardiomyopathy

2. These cardiomyopathies are characterized by lipid accumulation and are very sensitive to Car treatment.

3. Asynergy , however, occasionally has been observed also in primary cardiomyopathies (2-4), even if its sig- nificance in these diseases is not well understood

4. Such a classification of cardiomyopathies was proposed in 2006 by the American Heart Association (AHA), which took the rapid evolution of molecular genetics in cardiology into account.

5. More than 40 IEMs are reported to cause cardiomyopathy, including fatty acid oxidation defects, glycogen, lysosomal and perioxisome storage diseases, mitochondrial cardiomyopathies, organic acidaemias, aminoacidopathies and congenital disorders of glycosylation.