Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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The evolution of electrocardiographic changes in patients with Duchenne muscular dystrophies

  • Yoo, Woo Hyun (Department of Pediatrics, Biomedical Research Institute, Pusan National University Hospital) ;
  • Cho, Min-Jung (Department of Pediatrics, Biomedical Research Institute, Pusan National University Hospital) ;
  • Chun, Peter (Department of Pediatrics, Biomedical Research Institute, Pusan National University Hospital) ;
  • Kim, Kwang Hun (Department of Pediatrics, Biomedical Research Institute, Pusan National University Hospital) ;
  • Lee, Je Sang (Department of Rehabilitation Medicine, Biomedical Research Institute, Pusan National University Hospital) ;
  • Shin, Yong Beom (Department of Rehabilitation Medicine, Biomedical Research Institute, Pusan National University Hospital)
  • 투고 : 2016.11.22
  • 심사 : 2017.04.11
  • 발행 : 2017.06.15
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초록

Purpose: Myocardial dysfunction and dysrhythmias are inevitable consequences of Duchenne muscular dystrophy. We aimed to evaluate specific trends of electrocardiographic changes that reflect the progress of cardiomyopathy in patients with Duchenne muscular dystrophy. Methods: Fifty electrocardiograms (ECGs) of 30 patients (ages 1 to 27 years) who had not been prescribed medications for heart failure treatment at the time of examination were retrospectively analyzed and compared with 116 ECGs of age-matched healthy 116 controls. Heart rate, leads with fragmented QRS (fQRS), corrected QT, Tpeak-to-Tend, and Tpeak-to-Tend/QT were analyzed. Results: The patients with Duchenne muscular dystrophy failed to show a normal age-related decline in heart rate but showed an increasing trend in the prevalence of fQRS, corrected QT, corrected Tpeakto-Tend, and Tpeak-to-Tend/QT over time. In the ${\leq}10-year-old$ patient group, a significant difference was found only in the prevalence of fQRS between the patients and the controls. The prevalence of fQRS, heart rate, Tpeak-to-Tend/QT, and corrected Tpeak-to-Tend demonstrated significant differences between the patients and the controls in the middle age group (11 to 15 years old). All the indexes were statistically significantly different in the ${\geq}16-year-old$ patient group. Conclusion: The prevalence of lead with fQRS representing regional wall motion abnormalities was higher in the young patients than in the young healthy controls, and this might be one of the first signs of myocardial change in the patients. Markers of depolarization and repolarization abnormalities were gradually prominent in the patients aged >10 years. Further studies are needed to confirm these findings.

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참고문헌

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피인용 문헌

  1. Clinical utility of 12‐lead electrocardiogram in evaluating heart disease in patients with muscular dystrophy: Assessment of left ventricular hypertrophy, conduction disease, and cardiomyopathy vol.26, pp.6, 2017, https://doi.org/10.1111/anec.12876
  2. Low human dystrophin levels prevent cardiac electrophysiological and structural remodelling in a Duchenne mouse model vol.11, pp.1, 2021, https://doi.org/10.1038/s41598-021-89208-1