Primary myocardial disease (cardiomyopathies): a study of 69 cases.

Abstract

The clinical features, hemodynamic data and follow up of 69 children with cardiomyopathy (CM), up to the age of 12 years has been detailed. The diagnosis of CM was based on the report of WHO task force. Of the 69 children dilated CM was found in 64 cases whereas restrictive and hypertrophic cardiomyopathy were present in two and three patients respectively. The patients with dilated CM presented with congestive failure late in the course of the disease. Twenty-four of the 64 patients were found to have a mitral regurgitation murmur. The electrocardiogram was abnormal in each case; the common abnormalities being left or biventricular hypertrophy with nonspecific ST and T changes. Hemodynamic studies showed elevated right and left ventricular enddiastolic pressures and increased left ventricular volumes. In spite of clinical congestive failure right atrial pressure can be normal in infants with CM. Follow up was possible in 26 patients of whom 16 showed recovery. Of the 16 patients who showed recovery 10 have recovered completely (asymptomatic with normal heart size). The clinical, electrocardiographic radiological and hemodynamic data of patients who have recovered did not differ from those who did not show improvement. Vigorous anticongestive measures combined with bed rest was the main stay of treatment. The number of patients with restrictive and hypertrophic CM was too small for meaningful conclusions.