The outcome of idiopathic dilated cardiomyopathy and myocarditis in children from the west of Scotland

We analysed retrospectively all infants and children with idiopathic dilated cardiomyopathy (IDC) and myocarditis at the Regional Cardiac Centre of the Royal Hospital for Sick Children, Glasgow, during 1980–1997. Among the 39 patients with IDC, 25 (64%) were infants aged <1 year, eight (20.5%) had wheezing as the presenting symptom, and only six (15%) had a significant cardiac murmur. Thirty-eight of thirty-nine patients diagnosed in life were followed-up for 1 day to 15 years (median 3 years). Twelve of the thirty-nine (31%) died, six deaths were within a week of presentation and the rest within a year. The survival at 1 year and at 12 years was 0.69 (95% CI 0.54 to 0.84). Fourteen patients had histologically proven myocarditis, and all 9/14 (64%) detected at post-mortem and one of the five diagnosed in life died. Patients with myocarditis exhibited an actuarial survival of 0.29 (95% CI 0.04 to 0.53) at 1 year and at 9 years, significantly lower than IDC patients (log rank 9.8, P<0.01). There was no difference in the outcome for patients with positive or negative Coxsackie titres or who presented in the 1980s and in the 1990s. No risk factor that independently influenced the outcome or survival could be identified in either group. Thus our study from a relatively well-defined population of the west of Scotland showed that a significant proportion of children with IDC and myocarditis died in the first week of illness and that patients with myocarditis had shorter survival.