Myocardial biopsies have been obtained from patients with hypertrophic or congestive cardiomyopathies. Marker enzymes for the principal subcellular organelles of the myocardium were estimated using highly sensitive assay procedures. The results were compared with those obtained in tissue from patients with valvular heart disease with good or poor left ventricular function. Left ventricular myocardial tissue from patients with hypertrophic cardiomyopathy showed essentially normal levels of enzymic activities. In congestive cardiomyopathy, right ventricular tissue showed reduced levels of mitochondrial enzymes with increased levels of lactate dehydrogenase. Left ventricular tissue from patients with congestive cardiomyopathy showed reduced levels of mitochondrial and myofibril enzymes but high levels of lactate dehydrogenase. The reduced levels of myofibril Ca++-activated ATP in congestive cardiomyopathy is similar to that found in patients with impaired left ventricular function secondary to valvular disease. It is suggested that defective mitochondrial function is a characteristic feature of congestive cardiomyopathy and that the increased levels of lactate dehydrogenase reflect a compensatory response.
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