Related Experiment Video
Updated: Aug 14, 2026

Isolation and Functional Characterization of Human Ventricular Cardiomyocytes from Fresh Surgical Samples
Published on: April 21, 2014
Prognostic features of children with idiopathic dilated cardiomyopathy
H B Wiles1, P D McArthur, A B Taylor
1South Carolina Children's Heart Center, Medical University of South Carolina, Charleston 29425.
Insights
Idiopathic dilated cardiomyopathy (IDC) in children has unpredictable outcomes. Key presenting features like age or heart failure do not consistently predict long-term results for pediatric IDC patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Clinical Pediatrics
Background:
- Idiopathic dilated cardiomyopathy (IDC) is a significant cause of heart failure in children.
- Understanding prognostic factors is crucial for managing pediatric IDC.
- Long-term outcomes and predictors of disease progression in pediatric IDC remain incompletely defined.
Purpose of the Study:
- To review the presenting features and long-term outcomes of children diagnosed with idiopathic dilated cardiomyopathy.
- To identify clinical factors that may predict the final outcome in pediatric IDC patients.
- To inform appropriate management strategies for children with IDC.
Main Methods:
- Retrospective review of 39 children diagnosed with idiopathic dilated cardiomyopathy.
- Categorization of patients into four outcome groups: death, improvement, resolution, or transplant.
- Analysis of presenting clinical features, electrocardiogram findings (left ventricular hypertrophy, rhythm disturbance), and age at presentation for correlation with outcomes.
Main Results:
- Presenting features such as age, sex, congestive heart failure, cardiomegaly, and ventricular dysfunction did not predict outcome.
- Left ventricular hypertrophy on ECG was associated with improvement (p = 0.002).
- Rhythm disturbances were more frequent in patients who died (p = 0.025).
- 33% of medically treated patients died, 42% improved, and 25% resolved.
- Survival rates were higher for patients presenting at age ≤2 years (69%) compared to those >2 years (60%).
Conclusions:
- No single clinical feature, including age at presentation, consistently predicts the ultimate outcome in children with idiopathic dilated cardiomyopathy.
- Electrocardiographic findings like left ventricular hypertrophy and rhythm disturbances may offer some prognostic insights.
- The unpredictable nature of pediatric IDC necessitates careful monitoring and individualized management approaches.
Abstract:
The presenting features and long-term outcome of 39 children (median age 6.5 months, range 1 day to 16 years) with idiopathic dilated cardiomyopathy (IDC) were reviewed to help determine the appropriate management of these patients. Four outcome groups were identified: those who died, improved, had IDC resolved or received transplants. Presenting clinical features of age, sex, race, congestive heart failure, cardiomegaly, and degree of systolic ventricular dysfunction did not predict final outcome. Left ventricular hypertrophy on the electrocardiogram was seen significantly more often in children who improved than in those who died or in whom IDC resolved (p = 0.002). A rhythm disturbance was also seen more often in those who died than in those who survived (p = 0.025). Of 36 patients treated medically, 12 (33%) died, 15 (42%) improved and 9 (25%) resolved. Eighteen of 26 (69%) patients presenting at age less than or equal to 2 years survived, whereas 6 of 10 patients greater than 2 years survived. There were no differences based on age at presentation, in the time to death or time of follow-up. Three patients received orthotopic heart transplants, 1 of whom died from graft failure. Thus, no clinical feature including age at presentation consistently predicts ultimate outcome in children with IDC.
Related Concept Videos
Mitral Stenosis II: Clinical features and Diagnostic Tests
Cardiomyopathy I: Introduction and Classification
Cardiomyopathy II: Dilated Cardiomyopathy
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Cardiomyopathy IV: Restrictive Cardiomyopathy
Cardiomyopathy V: Interprofessional Care

