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Updated: Aug 23, 2026

Implantation of Total Artificial Heart in Congenital Heart Disease
Published on: July 18, 2014
Improved outcomes of pediatric dilated cardiomyopathy with utilization of heart transplantation
Anna E Tsirka1, Kathryn Trinkaus, Su-Chiung Chen
1Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri, USA.
Insights
Outcomes for pediatric dilated cardiomyopathy (DCM) vary, with recovery being most common. Heart transplantation significantly improves survival rates for these young patients.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Medical Outcomes Research
Background:
- Pediatric dilated cardiomyopathy (DCM) has a high mortality rate, with 50-60% of patients dying within five years.
- The long-term effects of heart transplantation and novel treatments on pediatric DCM outcomes remain unclear.
Purpose of the Study:
- To investigate the outcomes of pediatric patients diagnosed with dilated cardiomyopathy (DCM).
- To analyze the relationship between epidemiologic and echocardiographic variables and patient outcomes in pediatric DCM.
Main Methods:
- Retrospective study of 91 pediatric patients diagnosed with DCM between 1990 and 1999.
- Analysis of routine therapies including digoxin, diuretics, ACE inhibitors, and heart transplantation.
- Multivariate analysis to identify risk factors for adverse outcomes.
Main Results:
- At last follow-up, 36% of patients recovered left ventricular systolic function, 30% had persistent cardiomyopathy, 22% underwent heart transplantation, and 12% died without transplantation.
- Overall one-year survival was 90%, and five-year survival was 83%.
- Younger age (<1 year), older age (>12 years), and female gender were associated with increased risk of death or transplantation; higher left ventricular shortening fraction was associated with decreased risk.
Conclusions:
- Pediatric DCM patients exhibit diverse outcomes, with functional recovery being the most frequent positive result.
- Heart transplantation has demonstrably improved survival rates in pediatric DCM patients.
- Early identification of risk factors through echocardiography can inform management strategies.
Objectives:
We studied the outcomes of pediatric patients diagnosed with dilated cardiomyopathy (DCM) and their relation to epidemiologic and echocardiographic variables at the time of presentation.
Background:
The outcome of pediatric DCM patients ranges from recovery to a 50% to 60% chance of death within five years of diagnosis. The impact of heart transplantation and other emerging therapies on the outcomes of pediatric DCM patients is uncertain.
Methods:
We performed a retrospective study of the outcomes in 91 pediatric patients diagnosed with DCM from 1990 to 1999. Routine therapy included use of digoxin, diuretics, angiotensin-converting enzyme inhibitors, and heart transplantation.
Results:
At the time of last follow-up, 11 patients (12%) had died without transplantation; 20 (22%) underwent transplantation; 27 (30%) had persistent cardiomyopathy; and 33 (36%) had recovery of left ventricular systolic function. Overall actuarial one-year survival was 90%, and five-year survival was 83%. However, actuarial freedom from "heart death" (death or transplantation) was only 70% at one year and 58% at five years. Multivariate analysis found age <1 year (hazard ratio 7.1), age >12 years (hazard ratio 4.5), and female gender (hazard ratio 3.0) to be significantly associated with a greater risk of death or transplantation and a higher left ventricular shortening fraction at presentation (hazard ratio 0.92), with a slightly decreased risk of death or transplantation.
Conclusions:
Pediatric DCM patients continue to have multiple outcomes, with recovery of left ventricular systolic function occurring most frequently. Utilization of heart transplantation has led to improved survival after the diagnosis of pediatric DCM.
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