Electrocardiographic features of children with Duchenne muscular dystrophy
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 h...
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2022-08-01
|
| Series: | Orphanet Journal of Rare Diseases |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s13023-022-02473-9 |
| _version_ | 1798038805108752384 |
|---|---|
| author | Liting Tang Shuran Shao Chuan Wang |
| author_facet | Liting Tang Shuran Shao Chuan Wang |
| author_sort | Liting Tang |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-11T21:45:22Z |
| format | Article |
| id | doaj.art-e698f39a17e74368a1c9ad9c483d2bcd |
| institution | Directory Open Access Journal |
| issn | 1750-1172 |
| language | English |
| last_indexed | 2024-04-11T21:45:22Z |
| publishDate | 2022-08-01 |
| publisher | BMC |
| record_format | Article |
| series | Orphanet Journal of Rare Diseases |
| spelling | doaj.art-e698f39a17e74368a1c9ad9c483d2bcd2022-12-22T04:01:26ZengBMCOrphanet Journal of Rare Diseases1750-11722022-08-0117111610.1186/s13023-022-02473-9Electrocardiographic features of children with Duchenne muscular dystrophyLiting Tang0Shuran Shao1Chuan Wang2Department of Pediatric Cardiology, West China Second University Hospital, Sichuan UniversityDepartment of Pediatric Cardiology, West China Second University Hospital, Sichuan UniversityDepartment of Pediatric Cardiology, West China Second University Hospital, Sichuan UniversityAbstract 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.https://doi.org/10.1186/s13023-022-02473-9Duchenne Muscular dystrophyElectrocardiogramAgeGenotypeCardiac function |
| spellingShingle | Liting Tang Shuran Shao Chuan Wang Electrocardiographic features of children with Duchenne muscular dystrophy Orphanet Journal of Rare Diseases Duchenne Muscular dystrophy Electrocardiogram Age Genotype Cardiac function |
| title | Electrocardiographic features of children with Duchenne muscular dystrophy |
| title_full | Electrocardiographic features of children with Duchenne muscular dystrophy |
| title_fullStr | Electrocardiographic features of children with Duchenne muscular dystrophy |
| title_full_unstemmed | Electrocardiographic features of children with Duchenne muscular dystrophy |
| title_short | Electrocardiographic features of children with Duchenne muscular dystrophy |
| title_sort | electrocardiographic features of children with duchenne muscular dystrophy |
| topic | Duchenne Muscular dystrophy Electrocardiogram Age Genotype Cardiac function |
| url | https://doi.org/10.1186/s13023-022-02473-9 |
| work_keys_str_mv | AT litingtang electrocardiographicfeaturesofchildrenwithduchennemusculardystrophy AT shuranshao electrocardiographicfeaturesofchildrenwithduchennemusculardystrophy AT chuanwang electrocardiographicfeaturesofchildrenwithduchennemusculardystrophy |