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Published on: August 2, 2019
Survival Without Cardiac Transplantation Among Children With Dilated Cardiomyopathy
Rakesh K Singh1, Charles E Canter2, Ling Shi3
1Department of Pediatrics, University of California-San Diego and Rady Children's Hospital, San Diego, California.
Insights
Pediatric dilated cardiomyopathy (DCM) survival has improved over 20 years, with lower death rates in recent cases. This improvement is not linked to heart transplantation rates but other factors.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Research
- Clinical Outcomes
Background:
- Historically, heart transplantation was the primary driver of improved survival in children with dilated cardiomyopathy (DCM).
- Understanding long-term transplant-free survival is crucial for managing pediatric DCM.
Purpose of the Study:
- To determine transplant-free survival rates for children with DCM over a 20-year period.
- To identify clinical characteristics at diagnosis that predict mortality in pediatric DCM patients.
Main Methods:
- Children under 18 with DCM were analyzed from the Pediatric Cardiomyopathy Registry, divided into early (1990-1999) and late (2000-2009) cohorts.
- Competing risks and multivariable modeling were employed to assess cumulative incidence of death, transplant, and echocardiographic normalization.
- Factors associated with death were identified.
Main Results:
- The study included 1,953 children; 1,199 in the early cohort and 754 in the late cohort.
- While echocardiographic normalization and heart transplantation rates were similar between cohorts, the death rate was significantly higher in the early cohort (18% vs. 9%).
- Diagnosis in the earlier era independently predicted a higher risk of death (hazard ratio: 1.4).
Conclusions:
- Children diagnosed with DCM in the more recent era exhibit improved survival outcomes.
- This enhanced survival appears to be influenced by factors beyond heart transplantation, as transplant rates remained consistent.
- Further research into these other factors is warranted to optimize pediatric DCM management.
Background:
Studies of children with dilated cardiomyopathy (DCM) have suggested that improved survival has been primarily due to utilization of heart transplantation.
Objectives:
This study sought to determine transplant-free survival for these children over 20 years and identify the clinical characteristics at diagnosis that predicted death.
Methods:
Children <18 years of age with some type of DCM enrolled in the Pediatric Cardiomyopathy Registry were divided by year of diagnosis into an early cohort (1990 to 1999) and a late cohort (2000 to 2009). Competing risks and multivariable modeling were used to estimate the cumulative incidence of death, transplant, and echocardiographic normalization by cohort and to identify the factors associated with death.
Results:
Of 1,953 children, 1,199 were in the early cohort and 754 were in the late cohort. Most children in both cohorts had idiopathic DCM (64% vs. 63%, respectively). Median age (1.6 vs. 1.7 years), left ventricular end-diastolic z-scores (+4.2 vs. +4.2), and left ventricular fractional shortening (16% vs. 17%) at diagnosis were similar between cohorts. Although the rates of echocardiographic normalization (30% and 27%) and heart transplantation (24% and 24%) were similar, the death rate was higher in the early cohort than in the late cohort (18% vs. 9%; p = 0.04). Being in the early cohort (hazard ratio: 1.4; 95% confidence interval: 1.04 to 1.9; p = 0.03) independently predicted death.
Conclusions:
Children with DCM have improved survival in the more recent era. This appears to be associated with factors other than heart transplantation, which was equally prevalent in both eras. (Pediatric Cardiomyopathy Registry [PCMR]; NCT00005391).
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