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Idiopathic dilated cardiomyopathy in children: a single medical center's experience
Ken-Pen Weng1, Chu-Chuan Lin, Shi-Huei Huang
1Department of Pediatrics, Kaohsiung Veterans General Hospital, Taiwan.
Insights
Idiopathic dilated cardiomyopathy (DCM) in children has a poor prognosis, with many succumbing to heart failure while awaiting transplants. Arrhythmia and low ejection fraction predict poor outcomes in pediatric DCM patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Transplantation Medicine
Background:
- Idiopathic dilated cardiomyopathy (DCM) presents a significant challenge in pediatric care.
- Limited donor availability in Taiwan exacerbates poor prognoses for children with DCM.
Purpose of the Study:
- To review the clinical experience with pediatric idiopathic DCM.
- To identify prognostic factors influencing outcomes in affected children.
Main Methods:
- Retrospective analysis of 18 pediatric idiopathic DCM patients (1990-2004).
- Patients categorized into survival and mortality groups.
- Comparison of clinical and laboratory data between groups.
Main Results:
- 13 out of 18 pediatric patients died, primarily from heart failure while awaiting transplantation.
- Cumulative survival rates were 50% at 1 year and 28% at 4 years.
- Arrhythmia and reduced left ventricular ejection fraction were identified as predictors of poor outcomes.
Conclusions:
- Pediatric idiopathic DCM carries a generally poor prognosis.
- The scarcity of organ donors significantly contributes to mortality in pediatric transplant candidates.
- Increased organ donation is crucial to improve survival rates for children with DCM awaiting transplantation.
Background:
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.
Methods:
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.
Results:
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.
Conclusion:
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.
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