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

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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.

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Data availability

The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable request.

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Funding

This work was supported by the National Natural Science Foundation of China (82271981, 82202104) and Sichuan Science and Technology Program (2022NSFSC1494, 2023YFG0284), Universal Application Project of Health Commission of Sichuan Province (21PJ048), Clinical Research Finding of Chinese Society of Cardiovascular Disease (CSC) of 2019 (HFCSC2019B01), and Fundamental Research Funds for the Central Universities (SCU2020D4132).

Author information

Author notes

  1. Wei-feng Yuan and Li Yu contributed equally to this work.

Authors and Affiliations

  1. Department of Radiology, Key Laboratory of Birth Defects and Related Diseases of Women and Children of Ministry of Education, West China Second University Hospital, Sichuan University, 20# Section 3 South Renmin Road, Chengdu, 610041, China

    Wei-feng Yuan, Ke Xu, Rong Xu, Hang Fu, Yu Song, Zi-qi Zhou, Ting Xu, Ying-kun Guo & Hua-yan Xu

  2. Department of Medical Imaging, Clinical Medical College and The First Affiliated Hospital of Chengdu Medical College, Chengdu, China

    Wei-feng Yuan

  3. Department of Pediatric Cardiovascular Medicine, West China Second University Hospital, Sichuan University, Chengdu, China

    Li Yu

  4. Department of Rehabilitation, Key Laboratory of Birth Defects and Related Diseases of Women and Children of Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu, China

    Xiao-tang Cai

Authors
  1. Wei-feng Yuan
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  2. Li Yu
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Contributions

W.Y., L.Y., Y.G., and H.X. conceived, supervised, and supported the study. W.Y., L.Y., K.X., R.X., H.F., Y.S., Z.Z., T.X., and X.C. collated and analyzed the data, performed the statistical analysis, and drafted the initial manuscript. W.Y., L.Y., and H.X. interpreted the images. All authors reviewed and approved the final manuscript.

Corresponding author

Correspondence to Hua-yan Xu.

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Yuan, Wf., Yu, L., Xu, K. et al. Left ventricular concentric hypertrophy with cardiac magnetic resonance imaging improves risk stratification in patients with Duchenne muscular dystrophy: a prospective cohort study. Pediatr Radiol 54, 208–217 (2024). https://doi.org/10.1007/s00247-024-05856-1

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  • DOI: https://doi.org/10.1007/s00247-024-05856-1

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