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Imaging based risk factors for heart failure death in childhood dilated cardiomyopathy: a systematic review and
Christina Street-de Palma1, Zhia Lim1,2, Ella Field1,2
1Centre for Paediatric Inherited & Rare Cardiovascular Disease, Institute of Cardiovascular Science, London, United Kingdom.
Insights
This study identified key imaging markers for predicting heart failure in childhood dilated cardiomyopathy (DCM). Higher left ventricular end-diastolic diameter, lower ejection fraction or fractional shortening, and severe mitral regurgitation are significant risk factors.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Medical Imaging
Background:
- Dilated cardiomyopathy (DCM) is a leading cause of heart failure death in children.
- Prognostic imaging characteristics in pediatric DCM are not well-established.
- Predicting disease progression in childhood DCM is challenging.
Purpose of the Study:
- To systematically review and meta-analyze imaging characteristics associated with adverse outcomes in childhood DCM.
- To identify reliable imaging predictors of heart failure events in pediatric patients with DCM.
Main Methods:
- Systematic literature review and meta-analysis of studies published from 1946 to July 2024.
- Searched PubMed, Embase, and Scopus databases for English-language articles.
- Included studies with pediatric patients (<21 years) diagnosed with DCM and heart failure-related endpoints.
Main Results:
- Thirteen studies comprising 1,348 patients were analyzed.
- Identified significant imaging risk factors: increased left ventricular end-diastolic diameter Z-score (LVEDD), decreased left ventricular ejection fraction (LVEF), decreased LV fractional shortening (LVFS), and severe mitral regurgitation (MR).
- Fibrosis on cardiac MRI (CMRI) showed no significant association with adverse outcomes in limited studies.
Conclusions:
- Higher LVEDD, lower LVEF or LVFS, and severe MR are major imaging risk factors for adverse outcomes in childhood DCM.
- The findings underscore the need for robust multicenter studies to validate these imaging predictors.
- Improved imaging-based risk stratification can aid in managing pediatric DCM and preventing heart failure events.
Background And Aim:
Dilated cardiomyopathy (DCM) is the most common heart muscle disease presenting in childhood and is associated with an increased risk of heart failure related death. In adult cohorts, imaging characteristics such as increased left ventricular dimensions or the presence of fibrosis on cardiac magnetic resonance imaging (MRI) have been shown to be associated with adverse outcomes. In contrast, the prognostic relevance of imaging characteristics in childhood cohorts remains unclear and predicting disease progression is challenging. The aim of this study was to perform a systematic literature review and meta-analysis of imaging characteristics associated with adverse outcomes in childhood DCM.
Methods:
PubMed, Embase, and Scopus databases were searched for original articles published in English from 1946 to July 2024 that included patients under 21 years with a confirmed diagnosis of DCM and primary or secondary end-points of heart failure death or equivalent event (heart transplantation or ventricular assist device implantation). Studies were excluded if imaging characteristics were not described.
Results:
Thirteen studies (1,348 patients) met the inclusion criteria. All but one study was retrospective and only one had a patient cohort of more than 100 patients. We identified four imaging risk factors that were evaluated in at least four studies and significantly associated with a heart failure end point in at least two; left ventricular end-diastolic diameter Z score (LVEDD) [pooled hazard ratio [HR] 1.43, 95% confidence interval [CI] 1.13-1.81, p = 0.003], left ventricular ejection fraction (LVEF) (pooled HR 0.8, 95% CI: 0.65-0.99, p = 0.04), LV fractional shortening (LVFS) and severe mitral regurgitation (MR) [pooled odds ratio (OR) 5.12, 95% CI: 1.18-22.19, p = 0.004]. Two small studies investigated the role of fibrosis on CMRI and did not report an association with adverse outcomes.
Conclusions:
A systematic review and meta-analysis of imaging risk factors predicting heart failure adverse events in childhood DCM was performed identifying three "major" risk factors; higher LVEDD, lower LVEF or LVFS and severe MR. The findings highlight a significant need for well-designed multicentre studies to investigate the role of imaging characteristics in predicting outcome in the paediatric population.
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