PREDICTORS OF DISEASE PROGRESSION IN IDIOPATHIC DILATED (CONGESTIVE) CARDIOMYOPATHY IN PEDIATRIC AGE

Abd El Mohsen A. M, Antonios M. A. and Abd-El-Fattah N. A.*

ABSTRACT

Idiopathic dilated cardiomyopathy (IDCM) is a severe illness which carries a high mortality rate in the pediatric population. In order to characterize IDCM evolution and identifyprognostic predictors in the pediatric cardiology outpatient clinic of Alexandria University Children’s Hospital, allpatients with IDCM (n=22) were evaluated clinically and by echocardiography. They were followed for one year. Patients less than 10 years represented 72.7% of the cohort (n=16). Gender distribution revealed 59.1% male (n=13) and 40.9 % female (n=9). Outcomes were divided into four groups: 13.6% of patients (n=3) cured, 27.3% of patients (n=6) compensated, 50%patients (n=11) decompensated and 9.1% ofpatients (n=2) died. In this study, we found a significant correlation of prognosis with ejection fraction, end diastolic dimension of left ventricle, and shortening fraction on presentation. Also results suggested that pulmonary congestion in chest x- ray at presentation was significantly related to unfavorable outcome. Medical treatment and good compliance were associated with a statistically better prognosis. We concluded that further multi-center studies arenecessary to verify predictors of outcome in IDCM patients. Identification of markers affecting early myocardialfunction is essential to achieving improvements in treatments and consequently outcomes in this pediatricpopulation.

Keywords: dilated cardiomyopathy, children, natural history, predictors of prognosis, echocardiography.