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Published on: May 16, 2020
Dilated cardiomyopathy in children and adults: what is new?
Galal E Nagib Elkilany1, Mustafa A Al-Qbandi, Khaled A Sayed
1Adult Cardiology Department, Echocardiography Laboratory, Chest Hospital, Safat, Kuwait. galal_elkilany@yahoo.com
Insights
Dilated cardiomyopathy (DCM) is a serious heart condition. Key risk factors for poor outcomes in children and adults include age, heart failure, and specific echocardiographic findings.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Cardiovascular Imaging
Background:
- Dilated cardiomyopathy (DCM) is the leading cause of heart transplantation in pediatric and young adult populations, with high associated mortality.
- Limited established data exists regarding mortality, clinical course, and echocardiographic characteristics of DCM in children versus adults.
- Understanding these factors is crucial for improving patient outcomes and management strategies.
Purpose of the Study:
- To provide a comprehensive research article detailing the incidence, causes, outcomes, and risk factors of DCM.
- To identify new echocardiographic criteria for assessing the risk of death in DCM patients.
- To establish a clearer understanding of DCM's diverse clinical presentation and prognostic indicators.
Main Methods:
- Retrospective analysis of pediatric and adult patient cohorts diagnosed with DCM.
- Detailed echocardiographic assessments including ejection fraction, global strain, diastolic function, and valvular competence.
- Statistical analysis to identify independent risk factors for mortality and need for cardiac transplantation or CRT.
Main Results:
- In children, independent risk factors for death/transplantation included older age, heart failure, low ejection fraction (≤25%), low global strain, mitral incompetence, pulmonary hypertension, diastolic dysfunction, and right ventricular involvement.
- In adults, risk factors included low ejection fraction (<30-35%), global peak systolic strain <-7.6%, increased end-diastolic volume (EDV), end-systolic volume (ESV), left bundle branch block (LBBB), diastolic dysfunction, and left ventricle dyssynchrony.
- All identified risk factors were statistically significant (p<0.001).
Conclusions:
- DCM is a heterogeneous disorder in both children and adults, with outcomes significantly influenced by the underlying cause, patient age, and heart failure status at presentation.
- Echocardiographic parameters, including left ventricular systolic and diastolic function, pulmonary artery pressure, global strain, and mitral valve function, are critical determinants of prognosis.
- This study offers novel insights into diagnostic criteria and risk stratification for DCM, paving the way for improved patient management.
Abstract:
Dilated cardiomyopathy (DCM) is the most common form of cardiomyopathy and cause of cardiac transplantation in children and young adults; mortality is high among this patient population. However, mortality, clinical course, and illustrative echocardiographic data of DCM in children and adults are not well established. Our objective was to provide a research article of detailed descriptions of the incidence, causes, outcomes, related risk factors, and new echocardiographic criteria of risk of death from DCM. Our results showed that independent risk factors at DCM diagnosis for subsequent death or transplantation in children cohorts were older age, congestive heart failure, lower left ventricular ejection fraction (EF< or =25%), low global strain, significant mitral valve incompetence, pulmonary hypertension, diastolic dysfunction, right ventricular involvement, and cause of DCM (p<0.001 for all). In adults, low ejection fraction (<30-35%), global peak systolic strain <-7.6%, increased EDV, ESV, LBBB, diastolic dysfunction, and left ventricle dyssynchrony were the main independent risk factors for major cardiac events and need for CRT or transplantation (p<0.001 for all). Our conclusions were that in children and adults, DCM is a diverse disorder with outcomes that depend largely on cause, age, heart failure status at presentation, and echocardiographic parameters of the heart (systolic and diastolic function of left ventricle, pulmonary artery pressure, global strain, and valvular function of the mitral valve). This study will present new findings in the diagnostic area.
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