The prognosis of patients with idiopathic dilated cardiomyopathy (DCM) is poor. Most patients die while waiting for cardiac transplantation because of the small number of donors in Taiwan. The purpose of this study was to review our experience with pediatric patients diagnosed with idiopathic DCM and attempt to discover prognostic factors.
Eighteen patients with idiopathic DCM presenting between 1990 and 2004 were identified. They were classified into 2 groups according to outcome: group 1 comprised 13 patients who died; group 2 comprised 5 who survived. Clinical findings and laboratory investigations were compared between the 2 groups.
The age at initial diagnosis for the 18 patients (11 males, 7 females) ranged from fetus to 13 years (median, 3 months). The follow-up period ranged from 12 days to 44 months (median, 7 months) in group 1, and from 1 to 48 months (median, 39 months) in group 2. Of the 18 patients, 13 (72%) died: 11 died from severe heart failure while waiting for cardiac transplantation. The cumulative survival rate was 50% at 1 year and 28% at 4 years. The presence of arrhythmia and low left ventricular ejection fraction were predictive of a poor outcome.
The diagnosis of idiopathic DCM in children is associated with a generally poor prognosis. The lack of available donors results in significant mortality for pediatric patients awaiting transplantation. Advocating organ donation to increase the size of the organ donor pool is needed to significantly reduce the mortality rate in such patients.