The cardiac function of 36 males with Duchenne muscular dystrophy was evaluated by echocardiography, and the results were compared with the results of other tests of cardiac involvement, including serum creatine kinase isoenzyme evaluation, electrocardiography, chest x-ray, and physical examination of the heart and lungs. Although correlation between the various methods of assessment was not impressive, the echocardiographic technique appeared to be useful in evaluating and following the cardiomyopathy in these patients. Several aspects of left ventricular function were abnormal in most patients, and only two of the 36 had normal left ventricular size and function. Electrocardiographic abnormalities were also apparent in all patients, although the changes were mild in four of the 36. Clinical evaluation, isoenzyme determinations, and chest x-rays have not been useful in assessing cardiac function. The echocardiographic data on these 36 patients are presented in detail and the patients will now be followed sequentially to attempt evaluation of ongoing changes.
This report presents an infant who was initially admitted with a pericardial effusion. Subsequent evaluation led to the diagnosis of a primary cardiac tumor. Excision of the tumor which arose from the atrial septum was possible only by removing most of the free right atrial wall and a portion of the septal leaflet of the tricuspid valve. Reconstruction of the tricuspid valve leaflet by reattachment of chordae and a partial annuloplasty was done with the right atrial wall being replaced using pericardial tissue. The histology of the tumor was compatible with a fibroma, a type not previously reported in this location in the pediatric age group. Twenty-one months following operation, the child was restudied and the catheterization revealed mild to moderate tricuspid regurgitation and no recurrence of the tumor. The child is now 4 years of age and remains asymptomatic with normal growth and development and no evidence of fibromatosis.
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