Prevalence and associated factors of myocardial involvement in Duchenne muscular dystrophy patients in the first decade of life

Rong Xu, Huayan Xu, Kun Zhang, Hong Xu, Hui Liu, Hang Fu, Linjun Xie, Ke Xu, Chuan Fu, Xuesheng Li, Xiaoyue Zhou, Rajiv Ananthakrishna, Joseph B. Selvanayagam, Li Yu, Xiaotang Cai, Yingkun Guo

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To the Editor: Duchenne muscular dystrophy (DMD) is a rare X-linked inherited disorder caused by dystrophin deficiency, which results in sarcolemmal fragility and degeneration of skeletal and cardiac myocytes. Cardiac involvement is manifested as DMD cardiomyopathy, which is the leading cause of disease-related morbidity and mortality among DMD patients. However, the signs and symptoms of cardiomyopathy frequently are subtle and often overlooked during the ambulatory and early non-ambulatory stages. Therefore, non-invasive imaging methods are recommended for the assessment of myocardial involvement of DMD patients in the updated diagnosis and management statement, and echocardiography (Echo) and cardiac magnetic resonance (CMR) are two of the most commonly used imaging methods. However, conventional Echo cannot evaluate the characteristics of myocardial tissue and plays a limited role in early diagnosis. CMR with multiple sequences and parameters allows detailed feature evaluation, and especially the evaluation of myocardial fibrosis by late gadolinium enhancement (LGE) is highly consistent with pathology. Previous studies have reported the assessment of myocardial fibrosis in DMD patients by LGE, but studies investigating the prevalence of myocardial fibrosis in DMD patients during the first decade of life is still lacking. Moreover, DMD is a multisystem disease, loss of dystrophy protein function could occur in skeletal muscle and cardiomyocytes. In practice, individuals with DMD start skeletal muscle management early but are not referred to a cardiac specialist until late in the disease. Therefore, it is important to clarify the association between skeletal muscle involvement and myocardial involvement in the early stages of disease, which may help clinicians start multidisciplinary management earlier and improve the quality of life of patients further. In the current study, the prevalence of LGE presence at the first decade life of DMD patients was described. Logistic regression analysis was used to determine the association factors of LGE presence...
Original languageEnglish
Pages (from-to)1132-1134
Number of pages3
JournalChinese Medical Journal
Issue number9
Early online date28 Mar 2023
Publication statusPublished - 5 May 2023


  • Duchenne muscular dystrophy (DMD)
  • Heart disease


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