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Published in: Pediatric Radiology 2/2024

25-01-2024 | Duchenne Muscular Dystrophy | Original Article

Left ventricular concentric hypertrophy with cardiac magnetic resonance imaging improves risk stratification in patients with Duchenne muscular dystrophy: a prospective cohort study

Authors: Wei-feng Yuan, Li Yu, Ke Xu, Rong Xu, Hang Fu, Yu Song, Zi-qi Zhou, Ting Xu, Xiao-tang Cai, Ying-kun Guo, Hua-yan Xu

Published in: Pediatric Radiology | Issue 2/2024

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Abstract

Background

The development of left ventricular (LV) remodeling has been associated with an increased cardiovascular risk and cardiogenic death, and different patterns of remodeling result in varying levels of prognosis.

Objective

To investigate the association between different patterns of LV remodeling and clinical outcomes in the preclinical stage of patients with Duchenne muscular dystrophy (DMD).

Materials and methods

A total of 148 patients with DMD and 43 sex- and age-matched healthy participants were enrolled. We used the four-quadrant analysis method to investigate LV remodeling based on cardiac magnetic resonance (MR) imaging. Kaplan-Meier curves were generated to illustrate the event-free survival probability stratified by the LV remodeling pattern. Cox regression models were constructed and compared to evaluate the incremental predictive value of the LV remodeling pattern.

Results

During the median follow-up period of 2.2 years, all-cause death, cardiomyopathy, and ventricular arrhythmia occurred in 5, 35, and 7 patients, respectively. LV concentric hypertrophy (hazard ratio 2.91, 95% confidence interval 1.47–5.75, P=0.002) was an independent predictor of composite endpoint events. Compared to the model without LV concentric hypertrophy, the model with LV concentric hypertrophy had significant incremental predictive value (chi-square value 33.5 vs. 25.2, P=0.004).

Conclusion

Age and late gadolinium enhancement positivity were positively correlated with clinical outcomes according to the prediction models. LV concentric hypertrophy was also an independent predictor for risk stratification and provided incremental value for predicting clinical outcomes in the preclinical stage of patients with DMD.

Graphical Abstract

Appendix
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Metadata
Title
Left ventricular concentric hypertrophy with cardiac magnetic resonance imaging improves risk stratification in patients with Duchenne muscular dystrophy: a prospective cohort study
Authors
Wei-feng Yuan
Li Yu
Ke Xu
Rong Xu
Hang Fu
Yu Song
Zi-qi Zhou
Ting Xu
Xiao-tang Cai
Ying-kun Guo
Hua-yan Xu
Publication date
25-01-2024
Publisher
Springer Berlin Heidelberg
Published in
Pediatric Radiology / Issue 2/2024
Print ISSN: 0301-0449
Electronic ISSN: 1432-1998
DOI
https://doi.org/10.1007/s00247-024-05856-1

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