Restrictive Cardiomyopathy in Children: A Clinicopathologic Study in 12 Cases

Clinical and laboratory findings were compared with the clinical course and prognosis in 12 children with restrictive cardiomyopathy. The mean age of the patients was 5.0 years. The most common symptoms and clinical findings were fatigue, abdominal distension, exertional dyspnea and hepatomegaly. ECG demonstrated evidence of atrial dilatation and telecardiogram showed cardiomegaly in all cases. Echocardiography revealed left or biatrial dilatation. In eight cases who underwent cardiac catheterization, atrial mean pressures and ventricular end-diastolic pressures were above normal. Endomyocardial biopsy in six cases showed thickening of the endocardium, hypertrophy in myocardial fibers and interstitial fibrosis.