Dilated cardiomyopathy (DCM) is a rare disease in the paediatric population. We analysed the epidemiology, clinical features and role of immunotherapy in the treatment of myocarditis. On the basis of experimental evidence, indicating that autoimmunity might play a role in the development of myocarditis, we treated children affected by myocarditis with immunosuppressive therapy, and we present here our series. The future availability of reliable prognostic markers should allow treatment of only those children with myocarditis who do not spontaneously recover. The possibility that DCM with myocarditis is a distinct pathological entity from the non-inflammatory form of DCM is suggested.
Conclusion: The high long-term survival rate observed in our children with myocarditis is probably due to the effect of short-term immunosuppression. This result is at odds with previously published series of conventionally treated children, whose survival probability at 1 y was approximately 0.60.