Electrocardiographic features of children with Duchenne muscular dystrophy

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作者
Liting Tang
Shuran Shao
Chuan Wang
机构
[1] Sichuan University,Department of Pediatric Cardiology, West China Second University Hospital
[2] Sichuan University,The Cardiac Development and Early Intervention Unit, West China Institute of Women and Children’s Health, West China Second University Hospital
[3] West China Medical School of Sichuan University,Key Laboratory of Development and Diseases of Women and Children of Sichuan Province, West China Second University Hospital
[4] Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University),undefined
[5] Ministry of Education Chengdu,undefined
[6] Sichuan University,undefined
关键词
Duchenne Muscular dystrophy; Electrocardiogram; Age; Genotype; Cardiac function;
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学科分类号
摘要
Duchenne muscular dystrophy (DMD) is a clinically common X-linked recessive myopathy, which is caused by mutation of the gene encoding dystrophin on chromosome Xp21. The onset of heart injury in children with DMD is inconspicuous, and the prognosis is poor once it develops to the stage of heart failure. Cardiovascular complications remain an important cause of death in this patient population. At present, population and animal studies have suggested that Electrocardiogram (ECG) changes may be the initial manifestation of cardiac involvement in children with DMD. Relevant clinical studies have also confirmed that significant abnormal ECG changes already exist in DMD patients before cardiomegaly and/or LVEF decrease. With increases in age and decreases in cardiac function, the proportion of ECG abnormalities in DMD patients increase significantly. Some characteristic ECG changes, such as ST-segment changes, T wave inversion, Q wave at the inferolateral leads, LBBB and SDANN, have a certain correlation with the indexes of cardiac remodeling or impaired cardiac function in DMD patients, while VT and LBBB have demonstrated relatively good predictive value for the occurrence of long-term DCM and/or adverse cardiovascular events or even death in DMD patients. The present review discusses the electrocardiographic features in children with DMD.
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