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The prognosis for many children with idiopathic dilated cardiomyopathy is poor. The condition is an enlargement of the heart without apparent cause. Researchers in Finland studied 62 children with the condition. Half died, 6.4% underwent heart transplantation, and 16% recovered completely. Patients with signs of failure of the right ventricle, and those with fibroelastosis of the lining of the heart chambers, had the worst outcomes., 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 ([+ or -] TSD) follow-up of 3.9 [+ or -] 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 ([is less than] 1 year of age) and adolescent ([is 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. Pediatrics 1998,101:369-376; cardiomyopathy; congestive, child, adolescent, prognosis., Dilated cardiomyopathy (DCM) is a heterogeneous group of myocardial diseases characterized by cardiac dilatation and impaired myocardial contractility.[1] Despite an intensive search for causes using the most advanced molecular methods, [...]

Although idiopathic cardiomyopathies are prognostically important and are a common indication for cardiac transplantation in all age groups, the incidence and age distribution of idiopathic cardiomyopathies in a well-defined pediatric population have been poorly characterized. A retrospective study was carried out in Finland in 1980–1991 to obtain information on the epidemiology of childhood cardiomyopathies. The medical records of all patients aged birth to 20 years with cardiomyopathy from the five university hospitals and 16 central hospitals covering the entire country were reviewed. Moreover, data on causes of death from the Finnish National Census Bureau were examined. Of the 808 potential cases screened, 118 infants, children, and adolescents, representing an average age-specific population of 1.4 million, were definitely identified as having idiopathic cardiomyopathy. The average annual occurrence of new cases was 0.65 per 100,000 population (95% confidence interval (CI) 0.53–0.79). If the 15 cases diagnosed only after death during the 12-year study period were included, the occurrence increased to 0.74 per 100,000 population per year. Fifty-six new cases of dilated cardiomyopathy and 40 new cases of hypertrophic cardiomyopathy were diagnosed during the study period, giving average annual occurrences of 0.34/100,000/year (95% CI 0.26–0.44) and 0.24/100,000/year(95% CI 0.17–0.33) for new cases of dilated and hypertrophic cardiomyopathies, respectively. At the end of 1991, the prevalence of dilated cardiomyopathy was 2.6/100,000 (95% CI 1.8–3.6) and that for hypertrophic cardiomyopathy was 2.9/1 00,000 (95% CI 2.0–4.0). The number of new cases of dilated cardiomyopathy per year increased over the study period, whereas the annual occurrence of hypertrophic cardiomyopathy remained relatively constant. Marked variability was seen in occurrence among the different age groups of children with dilated cardiomyopathy, suggesting that different pathophysiologic mechanisms, and possibly etiologies, may exist in different age groups. Am J Epidemiol 1997;146:385–93. [ABSTRACT FROM AUTHOR]

Viral infections are the most common causes of myocarditis in children. Chronic myocardial injury may develop following an immune or autoimmune reaction triggered or maintained by an infection, or can be part of a systemic autoimmune disease. Although many of the children having developed myocarditis are symptomless, initial symptoms may include cardiac insufficiency, arrhythmias and sudden death. The diagnosis requires a clinical suspicion as well as laboratory and imaging studies. Recovery from myocarditises often takes place spontaneously, but some result in the development of dilated cardiomyopathy (DCM).