IDIOPATHIC DILATED CARDIOMYOPATHY IN CHILDREN - PROGNOSTIC INDICATORSAND OUTCOME

Objective. To determine the outcome of Finnish children and adolescent s with idiopathic dilated cardiomyopathy (IDCM) and factors that might be useful as prognostic indicators. Methodology. The clinical profile and course of 62 Finnish children and adolescents (median age, 13 mon ths; range, 1 day to 20 years) with IDCM in 1980 to 1991 were evaluate d to detect factors that might predict outcome. Factors studied includ ed age, gender, family history, previous viral illness, and symptoms a nd signs at presentation. Furthermore, data on serial electrocardiogra phic, echocardiographic, and chest x-ray examinations, histologic find ings, and treatments were analyzed. Results. During a mean (+/-SD) fol low-up of 3.9 +/- 4.5 years (range, 1 day to 25 years), 10 patients (1 6%) recovered, 17 (27%) had residual disease, 4 (6.4%) underwent heart transplantation, and 31 (50%) died. Infants (<1 year of age) and adol escent (greater than or equal to 15 years of age) male patients with p rogressing symptoms of left ventricular failure after initiation of me dical therapy tended to have the poorest outcome. However, in multivar iate analysis, only histologic evidence of endocardial fibroelastosis, clinical signs of right ventricular failure at presentation, and the need for anticoagulative therapy during follow-up, the last an express ion of a severely impaired left ventricular systolic function, appeare d to be significant predictors of long-term outcome. Conclusions. Our study confirms that the outcome of children with IDCM still remains po or. However, a group of patients, mainly infants, make a full recovery . Adolescent male patients as well as infants suffering from endocardi al fibroelastosis with persisting symptoms of congestive heart failure after initiation of medical therapy tend to have the poorest outcome. These patients need a careful follow-up at short time intervals and, in the case of lacking response to medical treatment with resulting gr owth failure and/or poor quality of life, should be offered urgent hea rt transplantation.