Dilated cardiomyopathy in childhood: prognostic features and outcome

The natural history of dilated cardiomyopathy in children is difficult to predict due to the heterogeneous character of the disease. The outcome in infants and children is highly variable from complete recovery to death. In this study, 40 children diagnosed with dilated cardiomyopathy between 1995 and 2004 in our paediatric cardiology unit were reviewed with respect to clinical course and outcome, retrospectively. The medical history of these patients with dilated cardiomyopathy was reviewed to determine age, gender, family history, preceding viral illness, duration of symptoms before the diagnosis, symptoms and signs at presentation, treatment and outcome. The diagnosis was made on the basis of cardiomegaly and evidence of poor left ventricular function by echocardiography. Median age at diagnosis was 14 months, ranging from 2 months to 8 years. At presentation, 28 patients (70%) were under and twelve (30%) were above the age of two years. Twenty-eight (70%) patients had signs of congestive heart failure. Mean duration of follow-up was 40 +/- 24 months (ranging from 6 months to 9 years), 21 patients (52.5%) recovered, 17 patients (42.5%) had residual disease and two (5%) died. The cause of death in both patients was progressive cardiac failure. Sixteen of 28 patients (57%) who were below the age of two years and five of 12 patients (42%) who were above the age of two years at presentation recovered. The rate of recovery was significantly different between the two age groups (p < 0.05). Seventeen of 21 (81%) patients with a history of recent viral illness at presentation recovered. The mean duration of the disease among those who recovered was 11 +/- 8.3 months. Five of 19 (26%) patients without recent viral illness recovered. The mean duration of the disease in this group was 22 +/- 12 months. There was a significant difference between the two groups with respect to recovery and recovery time (p < 0.05). During the first 6 months after diagnosis, there was a significant difference between the patients who recovered and the patients who had residual disease with respect to improvement in the left ventricular FS (22 +/- 3.5%, 15.2 +/- 2.8%, respectively) (p < 0.05). In conclusion, in this study, the rate of recovery and survival is higher than in previous studies. A good outcome is related to age at presentation (< or = two years old), a history of viral disease within three months of presentation and improvement in ventricular function during the first 6 months after diagnosis. Intractable heart failure has an adverse effect on the outcome.