Pediatric Cardiology

, Volume 38, Issue 5, pp 1042–1048 | Cite as

Relationship Between Fragmented QRS Complexes and Cardiac Status in Duchenne Muscular Dystrophy: Multimodal Validation Using Echocardiography, Magnetic Resonance Imaging, and Holter Monitoring

  • Min-Jung Cho
  • Ji-Won Lee
  • JeSang Lee
  • Yong Bum Shin
  • Hyoung Doo Lee
Original Article


The presence of fragmented QRS is a known marker of heterogeneous ventricular activation around the myocardial scar area. We validated whether fragmented QRS shows any association with ventricular dysfunction, fibrosis, or ventricular arrhythmias in patients with Duchenne muscular dystrophy (DMD). Thirty-seven patients with DMD were evaluated using electrocardiography (ECG), echocardiography, cardiac magnetic resonance, and 24-h Holter monitoring. Associations between fragmented QRS and ventricular dysfunction, fibrosis, or ventricular arrhythmia were investigated. Fragmented QRS complexes were present in 31 of 37 (83.7%) patients, and they were associated with a significantly lower left ventricular ejection fraction along with an increased left ventricular Tei index as evaluated by echocardiography, and more frequent ventricular arrhythmia as indicated using 24-hour Holter monitoring compared with patients without fragmented QRS. The number of leads with fragmented QRS correlated negatively with left ventricular ejection fraction both using echocardiography (r = −0.616) and CMR (r = −0.516). Further, the number of leads with fragmented QRS showed a significant correlation with several other echocardiographic measurements (mitral Em and Sm, and left ventricular Tei index), and 2-dimensional speckle-tracking echocardiography derived global left ventricular longitudinal strain. The frequency of ventricular arrhythmia observed using Holter monitoring showed a significant positive correlation with the frequency of fragmented QRS on ECG (r = 0.674). There was a positive trend of correlation between fragmented QRS and the amount of myocardial fibrosis as assessed by late gadolinium enhancement using CMR, but the statistical significance of the relationship was low (r = 0.433, p = 0.056). Fragmentation of QRS complexes is associated with degrees of left ventricular dysfunction, fibrosis, and ventricular arrhythmias in patients with DMD.


Muscular dystrophy Cardiomyopathy QRS fragmentation Electrocardiography Ambulatory Echocardiography Cardiac magnetic resonance 



This study was funded by Biomedical Research Institute Grant (2015-06), Pusan National University Hospital.

Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflict of interest to disclose.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed consent was obtained from all individual participants included in the study.


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Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  • Min-Jung Cho
    • 1
  • Ji-Won Lee
    • 2
  • JeSang Lee
    • 3
  • Yong Bum Shin
    • 3
  • Hyoung Doo Lee
    • 4
  1. 1.Department of Pediatrics, Biomedical Research Institute of Pusan National University HospitalPusan National University HospitalBusanRepublic of Korea
  2. 2.Department of RadiologyPusan National University HospitalBusanRepublic of Korea
  3. 3.Department of Rehabilitation MedicinePusan National University HospitalBusanRepublic of Korea
  4. 4.Pusan National University Children’s HospitalBusanRepublic of Korea

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