Dilated cardiomyopathy in Egyptian children: prevalence, clinical, echocardiographic and doppler profile

ABSTRACT

The prevalence and clinical profile of dilated cardiomyopathy [DC] in children seen at 2 large medical centers in Cairo were studied. Incidence was estimated from a retrospective analysis of 1 year data. A 4-year prospective study included 71 children with DC and aimed at defining the clinical profile, echocardiographic and hemodynamic characteristics, and prognosis. Doppler studies were done in 12 patients and cardiac catheterization in 14. An incidence of 33 per 100,000 children per year was obtained. From follow-up and mean age at presentation, a poor prognosis could be confirmed. Four-year mortaility was 82%. Left-sided failure was present in all children, while right-sided failure was seen in one half. Echocardiographic features included severe left ventricular [LV] dilatation, and decreased ejection fraction [mostly moderate-to-severe] in all children. Right ventricular [RV] dilatation was detected in 39.4%. Doppler evaluation in 12 cases showed mitral regurgitation in 66.6% and pulmonary hypertension in 75%. Cardiac catheterisation in 14 cases revealed elevated LV end-diastolic pressure in all children studied. Pulmonary artery and RV pressure were elevated in 93%