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Published on: July 14, 2021
Progressive Left Ventricular Remodeling for Predicting Mortality in Children With Dilated Cardiomyopathy: The
Paul F Kantor1, Ling Shi2, Steven D Colan3
1Children's Hospital Los Angeles and Keck School of Medicine of USC Los Angeles CA.
Insights
Changes in left ventricular (LV) function and size over time can predict outcomes in children with dilated cardiomyopathy. Improved LV fractional shortening (LVFS) and reduced LV dilation indicate better prognosis, highlighting the importance of serial echocardiographic monitoring.
Area of Science:
- Pediatric Cardiology
- Cardiovascular Imaging
- Clinical Outcomes Research
Background:
- Pediatric dilated cardiomyopathy (DCM) is a significant cause of mortality and heart transplantation in children.
- Predicting adverse outcomes in pediatric DCM is crucial for timely intervention and management.
Purpose of the Study:
- To investigate if changes in left ventricular (LV) end-diastolic dimension (LVEDD), LV end-diastolic posterior wall thickness, and LV fractional shortening (LVFS) over time can predict adverse outcomes in children with DCM.
Main Methods:
- Analysis of echocardiographic data from the Pediatric Cardiomyopathy Registry (1990-2009) for children with DCM.
- Assessment of changes in LV parameters (LVFS, LVEDD, wall thickness, and their ratio) between baseline and 1-year follow-up.
- Correlation of these changes with outcomes including death, cardiac transplantation, and survival at up to 7 years.
Main Results:
- Survivors showed improved LVFS and reduced LVEDD Z-scores, along with increased LV end-diastolic posterior wall thickness:LVEDD ratio, indicating reverse LV remodeling.
- Worsening LV dilation and stable or declining LVFS were observed in non-survivors.
- Improved LVFS at 1 year was associated with a lower risk of death or transplantation (HR 0.83), while progressive LV dilation increased this risk (HR 1.45).
Conclusions:
- Progressive decline in LV contractile function and increasing LV dilation are linked to mortality in pediatric DCM.
- Serial echocardiographic monitoring is essential for assessing disease progression and predicting outcomes in children with DCM.
Background:
Pediatric dilated cardiomyopathy often leads to death or cardiac transplantation. We sought to determine whether changes in left ventricular (LV) end-diastolic dimension (LVEDD), LV end-diastolic posterior wall thickness, and LV fractional shortening (LVFS) over time may help predict adverse outcomes.
Methods And Results:
We studied children up to 18 years old with dilated cardiomyopathy, enrolled between 1990 and 2009 in the Pediatric Cardiomyopathy Registry. Changes in LVFS, LVEDD, LV end-diastolic posterior wall thickness, and the LV end-diastolic posterior wall thickness:LVEDD ratio between baseline and follow-up echocardiograms acquired ≈1 year after diagnosis were determined for children who, at the 1-year follow-up had died, received a heart transplant, or were alive and transplant-free. Within 1 year after diagnosis, 40 (5.0%) of the 794 eligible children had died, 117 (14.7%) had undergone cardiac transplantation, and 585 (73.7%) had survived without transplantation. At diagnosis, survivors had higher median LVFS and lower median LVEDD Z scores. Median LVFS and LVEDD Z scores improved among survivors (Z score changes of +2.6 and -1.1, respectively) but remained stable or worsened in the other 2 groups. The LV end-diastolic posterior wall thickness:LVEDD ratio increased in survivors only, suggesting beneficial reverse LV remodeling. The risk for death or cardiac transplantation up to 7 years later was lower when LVFS was improved at 1 year (hazard ratio [HR], 0.83; P=0.004) but was higher in those with progressive LV dilation (HR, 1.45; P<0.001).
Conclusions:
Progressive deterioration in LV contractile function and increasing LV dilation are associated with both early and continuing mortality in children with dilated cardiomyopathy. Serial echocardiographic monitoring of these children is therefore indicated.
Registration:
URL: https://www.clinicaltrials.gov; Unique identifier: NCT00005391.
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