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Patients with dilated cardiomyopathy (DCM) have a large, poorly contractile, left ventricle. The causes of DCM are unknown but viral infection and/or autoimmunity are the favourite suggestions. Endomyocardial biopsy (EMB) studies have suggested two types of DCM; those with mononuclear infiltration of the myocardium (myocarditis) and those with only fibrosis (cardiomyopathy). With conventional non-immunosuppressive, treatment the outlook for these children has been poor with reported survival rates of 40–75% at 1 year and 37–47% at 5 years. Children, but not adults, with myocardial fibrosis tend to do worse than those with myocarditis and there is evidence that immunosuppressive treatment may be more effective in patients with myocarditis. Researchers in Rome (
) have reported a long-term observational study. Between March 1986 and December 2001 a total of 114 children (mean age 3 years 1 month, range <1 month to 19 years 9 months) were referred to the department with recent onset DCM and congestive heart failure. Heart defects and metabolic causes of DCM were excluded and all had EMB. The histological classification was: acute florid myocarditis (35), borderline myocarditis (35), and non-inflammatory cardiomyopathy (44). Children in the two myocarditis groups were treated with prednisone and cyclosporine in addition to conventional treatment whereas those with cardiomyopathy received only conventional treatment. The actuarial probability of event-free survival at 1 year was 0.96 in the myocarditis groups and 0.61 in the cardiomyopathy group. The probabilities of event-free survival at 13 years were 0.83 and 0.32. No patient in the acute florid myocarditis group died. Six in the borderline myocarditis group and three in the cardiomyopathy group died without heart transplant. Heart transplantation was performed in 32 patients; one with acute myocarditis, four with borderline myocarditis, and 27 with cardiomyopathy. Ten of the 27 died after transplantation. Complete recovery of cardiac function occurred in 79%, 64%, and 36% of survivors in the three groups respectively. Three factors predicted poor outcome: reduced left ventricular ejection fraction, increased left ventricular end diastolic volume, and non-inflammatory histology. Serial biopsies often showed resolution of the inflammatory changes in the children with myocarditis.
The children treated with immunosuppression in this series appeared to do better than children in previously reported series not given immunosuppression. The authors of this paper believe that a randomised trial might be unethical.