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Clinical profile of congestive cardiomyopathy in children
1St. Louis University School of Medicine, Department of Pediatrics, Cardinal Glennon Children's Hospital, Missouri 63104.
Insights
Congestive cardiomyopathy in children has a poor prognosis, with a 5-year mortality rate of 44%. Low shortening fraction, familial history, and endocardial fibroelastosis are key indicators of poor survival in pediatric patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Clinical Medicine
Background:
- Congestive cardiomyopathy presents a significant challenge in pediatric care.
- Identifying predictive factors for survival is crucial for managing affected children.
Purpose of the Study:
- To review the clinical profiles of children with congestive cardiomyopathy.
- To identify factors predicting survival in this pediatric population.
Main Methods:
- Retrospective review of 23 children diagnosed with congestive cardiomyopathy.
- Analysis of clinical data including age at onset, gender, radiographic, ECG, echocardiographic, hemodynamic, and biopsy findings.
- Long-term follow-up ranging from 1 month to 14 years.
Main Results:
- The 5-year mortality rate was 44%.
- Nonpredictive factors included age at onset, gender, cardiothoracic ratio, infarction patterns, ECG changes, and left ventricular end-diastolic pressure.
- Low shortening fraction (p < 0.01), familial cardiomyopathy, and endocardial fibroelastosis were associated with a poor prognosis.
Conclusions:
- Congestive cardiomyopathy in children carries a high mortality risk.
- Echocardiographic shortening fraction is a critical predictor of survival.
- Familial cardiomyopathy and endocardial fibroelastosis signify a very poor prognosis in pediatric cases.
Abstract:
The clinical profile of 23 children with congestive cardiomyopathy was reviewed to detect any factors that might be predictive for their survival. Factors examined include age at onset (less than 2 versus greater than 2 years), gender, severity of the clinical picture including data from the chest radiograph, electrocardiogram (ECG), echocardiogram, hemodynamic study and endomyocardial biopsy. Follow-up study ranged from 1 month to 14 years (mean 43 months). There were 12 survivors and 11 nonsurvivors; the 1 year mortality rate was 30% (7 of 23), and the 5 year mortality rate was 44% (10 of 23). Age at onset, gender, cardiothoracic ratio on chest radiograph, pattern of infarction, ST-T changes or arrhythmia on ECG and left ventricular end-diastolic pressure were nonpredictive of outcome. However, low shortening fraction (mean 11.5% in nonsurvivors versus 20.9% in survivors, p less than 0.01), familial cardiomyopathy and endocardial fibroelastosis indicated a very poor prognosis.