Long-term echocardiographic assessment of dilated cardiomyopathy in children

Left ventricular (LV) dimensions and function were assessed by echocardiography in 22 children with dilated cardiomyopathy. They had survived an initial episode of congestive heart failure in infancy for greater than or equal to 2 years. At the time of echocardiography, when they were 3 to 16 years old, 8 patients (Group 1) still had signs of dilated cardiomyopathy and 14 (Group 2) had lost all roentgenographic and electrocardiographic evidence of heart disease. All 8 patients in Group 1 (average follow-up 4.5 years) had significantly increased LV dimensions. The end-diastolic dimension averaged 144 +/- 18% of the normal value. Fractional LV shortening with systole was significantly reduced and averaged 23 +/- 3%. The E point-septal separation ranged from 7 to 17 mm (mean 12 +/- 4) and was far above the normal limit in all. Of the 14 patients in Group 2, seven (average follow-up 7 years) had normal ventricular dimensions and 7 (average follow-up 10 years) had LV dimensions larger than the upper range of the 95% prediction limit. In 6 of the latter patients the fractional LV shortening with systole was less than or equal to 31% and the E point-septal separation in excess of the upper limit of normal. These findings indicate that about half of the patients who had apparently recovered still had residual lesions as judged from the echocardiogram. In 6 patients in group 1, two-dimensional echo-cardiography allowed the visualization of a thickened endocardium. One of these 6 patients died. The echocardiographic image correlated well with the process of LV endocardial fibroelastosis found at necropsy.