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Published on: June 12, 2020
Electrocardiographic features of children with Duchenne muscular dystrophy
Liting Tang1,2, Shuran Shao1,2, Chuan Wang3,4,5,6
1Department of Pediatric Cardiology, West China Second University Hospital, Sichuan University, No. 20, 3rd section, South Renmin Road, Chengdu, 610041, Sichuan, China.
Insights
Electrocardiogram (ECG) changes are often the first sign of heart issues in children with Duchenne muscular dystrophy (DMD). These ECG abnormalities can predict future cardiac problems and death in DMD patients.
Area of Science:
- Cardiology
- Genetics
- Neurology
Background:
- Duchenne muscular dystrophy (DMD) is an X-linked recessive myopathy caused by dystrophin gene mutations.
- Cardiovascular complications are a leading cause of mortality in DMD patients.
- Early cardiac injury in DMD is often subtle, with poor prognosis upon heart failure development.
Purpose of the Study:
- To review the electrocardiographic (ECG) features in children diagnosed with Duchenne muscular dystrophy.
- To highlight the role of ECG as an early indicator of cardiac involvement in DMD.
- To correlate specific ECG abnormalities with cardiac remodeling and function in DMD.
Main Methods:
- Literature review of population and clinical studies on DMD patients.
- Analysis of existing data on electrocardiographic findings in pediatric DMD.
- Correlation of ECG parameters with cardiac function and adverse events.
Main Results:
- ECG abnormalities are present in DMD patients before cardiomegaly or decreased LVEF.
- The prevalence of ECG abnormalities increases with age and declining cardiac function.
- Specific ECG changes (e.g., ST-segment changes, T wave inversion, LBBB, VT) correlate with cardiac remodeling and predict adverse cardiovascular outcomes.
Conclusions:
- Electrocardiogram (ECG) changes are crucial for early detection of cardiac involvement in DMD.
- Certain ECG findings, like VT and LBBB, have significant predictive value for long-term cardiac events and mortality in DMD.
- Regular ECG monitoring is essential for managing cardiovascular health in children with DMD.
Abstract:
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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