Idiopathic dilated cardiomyopathy in children: Prognostic indicators and outcome

Objective. To determine the outcome of Finnish children and adolescents 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 months; range, 1 day to 20 years) with IDCM in 1980 to 1991 were evaluated to detect factors that might predict outcome. Factors studied included age, gender, family history, previous viral illness, and symptoms and signs at presentation. Furthermore, data on serial electrocardiographic, echocardiographic, and chest x-ray examinations, histologic findings, and treatments were analyzed. Results. During a mean (+/-SD) follow-up of 3.9 +/- 4.5 years (range, 1 day to 25 years), 10 patients (16%) recovered, 17 (27%) had residual disease, 4 (6.4%) underwent heart transplantation, and 31 (50%) died. Infants (<1 year of age) and adolescent (greater than or equal to 15 years of age) male patients with progressing symptoms of left ventricular failure after initiation of medical therapy tended to have the poorest outcome. However, in multivariate 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 expression of a severely impaired left ventricular systolic function, appeared to be significant predictors of long-term outcome. Conclusions. Our study confirms that the outcome of children with IDCM still remains poor. However, a group of patients, mainly infants, make a full recovery. Adolescent male patients as well as infants suffering from endocardial 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 growth failure and/or poor quality of life, should be offered urgent heart transplantation.