Primary lipid cardiomyopathy.

In this communication, we describe an isolated, apparently congenital cardiomyopathy (CMP) characterized by the accumulation of stainable lipid in mitochondria of cardiomyocytes. This lesion, which we term primary lipid cardiomyopathy, has not been reported so far. The structural alteration was associated with progressive heart failure, leading to death at the age of 3 years, and with massive hypertrophy of myocardium. Lipid storage in heart muscle cells resulted in an impressive yellow to orange color of the myocardium. We suggest that this type of primary CMP may represent a new member within the group of mitochondrial CMPs. Possible pathogenic mechanisms are discussed.