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Natural history of dilated cardiomyopathy in children
T Akagi1, L N Benson, N E Lightfoot
1Department of Pediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Childhood dilated cardiomyopathy has a poor prognosis, with a 1-year survival rate of 41%. Higher cardiothoracic ratio and lower left ventricular ejection fraction at presentation indicate increased risk in pediatric heart failure patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Heart Failure in Children
Background:
- Childhood dilated cardiomyopathy presents with congestive heart failure and reduced left ventricular contractility.
- Understanding the natural history and risk factors is crucial for improving outcomes in pediatric patients.
Purpose of the Study:
- To assess the natural history and identify potential risk factors for childhood dilated cardiomyopathy.
- To evaluate prognostic indicators in young patients diagnosed with this condition.
Main Methods:
- Retrospective investigation of 25 pediatric patients diagnosed with dilated cardiomyopathy after age 2.
- Analysis of clinical presentation, echocardiographic parameters (cardiothoracic ratio, left ventricular ejection fraction), and survival rates.
- Comparison of factors between patients who died within 1 year and those who survived beyond 1 year.
Main Results:
- Patients with dilated cardiomyopathy had significantly higher cardiothoracic ratios (65.1% vs 57.1%) and lower left ventricular ejection fractions (31.3% vs 40.0%) if they died within 1 year.
- Despite intensive medical therapy, the 1-year actuarial survival rate was 41%, and the 3-year survival rate was 20%.
- Cardiothoracic ratio and left ventricular ejection fraction were significant predictors of short-term mortality.
Conclusions:
- Childhood dilated cardiomyopathy carries a poor prognosis, with high mortality rates even with current medical interventions.
- Early identification of risk factors, such as elevated cardiothoracic ratio and reduced ejection fraction, is vital.
- Alternative or early therapeutic strategies should be considered for high-risk pediatric patients to improve survival rates.
Abstract:
To assess the natural history and potential risk factors in childhood dilated cardiomyopathy, we investigated 25 patients (ages 9.6 +/- 4.4 years) who presented after they were 2 years old. All patients had symptoms of congestive heart failure and reduced contractility with a dilated left ventricle at presentation. Two factors at presentation were significantly different between patients who died less than 1 year after the presentation (n = 14) and those who survived for more than 1 year (n = 9); cardiothoracic ratio (65.1% +/- 6.8% vs 57.1% +/- 6.1%, p less than 0.01) and left ventricular ejection fraction (31.3% +/- 7.0% vs 40.0% +/- 6.2%, p less than 0.05). Irrespective of intensive medical therapy, dilated cardiomyopathy in children had a poor prognosis; the actuarial survival rate was 41% at 1 year and 20% at 3 years. Other forms of therapy should be considered in the early stages of dilated cardiomyopathy in this high-risk group.