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Power LC, Gusso S, Hornung TS, et al. Exercise Cardiac Magnetic Resonance Imaging in Boys With Duchenne Muscular Dystrophy Without Cardiac Disease. Pediatr Neurol. 2020;117:35-43doi: 10.1016/j.pediatrneurol.2020.12.011.
Power, L. C., Gusso, S., Hornung, T. S., Jefferies, C., Derraik, J. G. B., Hofman, P. L., & O'Grady, G. L. (2021). Exercise Cardiac Magnetic Resonance Imaging in Boys With Duchenne Muscular Dystrophy Without Cardiac Disease. Pediatric neurology, 11735-43. https://doi.org/10.1016/j.pediatrneurol.2020.12.011
Power, Lisa C, et al. "Exercise Cardiac Magnetic Resonance Imaging in Boys With Duchenne Muscular Dystrophy Without Cardiac Disease." Pediatric neurology vol. 117 (2021): 35-43. doi: https://doi.org/10.1016/j.pediatrneurol.2020.12.011
Power LC, Gusso S, Hornung TS, Jefferies C, Derraik JGB, Hofman PL, O'Grady GL. Exercise Cardiac Magnetic Resonance Imaging in Boys With Duchenne Muscular Dystrophy Without Cardiac Disease. Pediatr Neurol. 2021 Apr;117:35-43. doi: 10.1016/j.pediatrneurol.2020.12.011. Epub 2020 Dec 30. PMID: 33662889.
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Pediatr Neurol. 2021 Apr;117:35-43. doi: 10.1016/j.pediatrneurol.2020.12.011. Epub 2020 Dec 30.

Exercise Cardiac Magnetic Resonance Imaging in Boys With Duchenne Muscular Dystrophy Without Cardiac Disease.

Pediatric neurology

Lisa C Power, Silmara Gusso, Tim S Hornung, Craig Jefferies, José G B Derraik, Paul L Hofman, Gina L O'Grady

Affiliations

  1. Paediatric Neurology Department, Starship Children's Hospital, Auckland, New Zealand; Paediatric Endocrinology Department, Starship Children's Hospital, Auckland, New Zealand; Liggins Institute, University of Auckland, Auckland, New Zealand.
  2. Department of Exercise Sciences, University of Auckland, Auckland, New Zealand.
  3. Paediatric Cardiology Department, Starship Children's Hospital, Auckland, New Zealand.
  4. Paediatric Endocrinology Department, Starship Children's Hospital, Auckland, New Zealand.
  5. Liggins Institute, University of Auckland, Auckland, New Zealand; Department of Women's and Children's Health, Uppsala University, Uppsala, Sweden.
  6. Paediatric Endocrinology Department, Starship Children's Hospital, Auckland, New Zealand; Liggins Institute, University of Auckland, Auckland, New Zealand.
  7. Paediatric Neurology Department, Starship Children's Hospital, Auckland, New Zealand. Electronic address: [email protected].

PMID: 33662889 DOI: 10.1016/j.pediatrneurol.2020.12.011

Abstract

BACKGROUND: Duchenne muscular dystrophy is caused by mutations in the DMD gene, resulting in cardiomyopathy in all affected children by 18 years. Although cardiomyopathy is now the leading cause of mortality in these children, there is ongoing debate regarding timely diagnosis, secondary prevention, and treatment of this condition. The purpose of this study was to use exercise cardiac magnetic resonance imaging in asymptomatic young boys with Duchenne muscular dystrophy to describe their heart function and compare this with healthy controls.

METHODS: We studied 11 boys with Duchenne muscular dystrophy aged 8.6 to 13.9 years and 11 healthy age- and sex-matched controls.

RESULTS: Compared with the controls, boys with Duchenne muscular dystrophy had lower ejection fraction at rest (57% versus 63%; P = 0.004). During submaximal exercise, they reached similar peak tachycardia but increased their heart rate and cardiac output only half as much as controls (P = 0.003 and P = 0.014, respectively). End-systolic volume remained higher in boys with Duchenne muscular dystrophy both at rest and during exercise. When transthoracic echocardiography was compared with cardiac magnetic resonance imaging, 45% of the echocardiograms had suboptimal or poor views in the Duchenne muscular dystrophy group.

CONCLUSIONS: Boys with Duchenne muscular dystrophy had abnormalities in left ventricular systolic function that were exaggerated by exercise stress. Exercise cardiac magnetic resonance imaging is feasible in a select population of children with Duchenne muscular dystrophy, and it has the potential to unmask early signs of cardiomyopathy.

Copyright © 2021 Elsevier Inc. All rights reserved.

Keywords: Cardiac magnetic resonance imaging; Cardiomyopathy; Duchenne muscular dystrophy; Exercise; Heart failure; Stress

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