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Dilated cardiomyopathy in children: determinants of outcome
M Burch1, S A Siddiqi, D S Celermajer
1Hospital for Sick Children, London.
Insights
Older children with dilated cardiomyopathy face a worse prognosis. Lack of improvement in heart function indicates a poor outcome, suggesting early heart transplantation may be beneficial for these pediatric patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Clinical Outcomes
Background:
- The natural history of childhood dilated cardiomyopathy is not well understood.
- Previous studies show variable outcomes related to age at presentation.
- Sudden death is infrequent in pediatric dilated cardiomyopathy cases.
Purpose of the Study:
- To determine the outcome of childhood-onset dilated cardiomyopathy.
- To identify prognostic factors for stratification in pediatric patients.
- To evaluate the long-term prognosis of idiopathic dilated cardiomyopathy in children.
Main Methods:
- Retrospective analysis of 63 pediatric patients with idiopathic dilated cardiomyopathy (1979-1992).
- Utilized Kaplan-Meier method for survival curve construction.
- Analyzed age at presentation, echocardiographic indices, and clinical outcomes.
Main Results:
- Actuarial survival was 79% at 1 year and 61% at 5 years.
- Older age at presentation (>2 years) was a significant predictor of adverse outcome.
- Lack of improvement or deterioration in left ventricular echocardiographic indices correlated with mortality and transplantation.
Conclusions:
- Older age at presentation and persistent poor systolic function are linked to adverse outcomes in pediatric dilated cardiomyopathy.
- Early heart transplantation should be considered for children with these poor prognostic indicators.
- Children with persistent abnormal echocardiographic dimensions face a risk of late sudden death.
Objective:
To determine the outcome of dilated cardiomyopathy presenting in childhood and the features that might be useful for prognostic stratification.
Setting:
Supraregional paediatric cardiology unit.
Design:
Retrospective analysis.
Background:
The natural history of dilated cardiomyopathy in children is not well characterised. Previous studies have shown a variable relation between age at presentation and outcome, and sudden death has been infrequent.
Methods:
Retrospective study of 63 consecutive patients with idiopathic dilated cardiomyopathy presenting between 1979 and 1992. Survival curves were constructed by the Kaplan-Meier method.
Results:
Age at diagnosis ranged from 1 day to 15 years (median 12 months) and follow up ranged from 1 day to 13 years (median 19 months). Actuarial survival from presentation was 79% at one year (95% confidence interval (95% CI) 66%-88%) and 61% (44%-74%) at five years. Univariate analysis showed that mural thrombus, left ventricular end diastolic pressure > 20 mm Hg, and age at presentation > 2 years were predictors of adverse outcome, but on multivariate analysis only age at presentation was significant. Left ventricular echocardiographic indices either did not improve or deteriorated in 36 children (17 of whom died, four suddenly, and three were transplanted), partially improved in 16 (three of whom died, all suddenly), and returned to normal in 11 (all of whom have survived).
Conclusions:
Older age at presentation and lack of improvement in systolic function are associated with an adverse outcome, and early transplantation should be considered in these patients. There is a persistent risk of late sudden death in those children in whom echocardiographic dimensions remain abnormal.