Long term follow up of children with myocarditis treated by immunosuppression and of children with dilated cardiomyopathy

Abstract

Objective: To describe the treatment and long term outcome after immunosuppressive treatment of children with myocarditis.

Methods and results: 114 patients with newly diagnosed dilated cardiomyopathy were divided into three groups, according to the histological pattern: group A, acute myocarditis; group B, borderline myocarditis; and group C, non-inflammatory cardiomyopathy. Groups A and B were treated with cyclosporine and prednisone in addition to conventional treatment. Survivors of the whole cohort were analysed for 13 year transplant-free survival and assessed for left ventricular function. Event-free survival at 13 years was 97 (3)% for group A, 70 (8)% for group B, and 32 (7)% for group C (p < 0.0001). It was 96 (4)% at one year and 83 (5)% at 13 years for the cumulated myocarditis group (A and B). Cardiac function recovered completely in 79% of survivors in group A, 64% in group B, and 36% in group C. The rate of complete recovery in the cumulated group (A and B) was 70%.

Conclusions: The high long term survival rate of this cohort of children with myocarditis is probably due to the effect of short term immunosuppression. This result differs from previously published series of conventionally treated children, whose survival probability at one year was about 60%.

Figure 1

Plot of event-free survival probability for children with acute myocarditis (group A), borderline myocarditis (group B), and non-inflammatory dilated cardiomyopathy (group C). For groups B and C the adverse events (transplant or death) are concentrated almost entirely in the first two years after the initial diagnosis.