Left ventricular dysfunction in Duchenne muscular dystrophy

Details

• Alternative Title:
  Cardiol Young
• Personal Author:
  James, Katherine A. ; Gralla, Jane ; Ridall, Leslie A. ; Do, ThuyQuynh N. ; Czaja, Angela S. ; Mourani, Peter M. ; Ciafaloni, Emma ; Cunniff, Christopher ; Donnelly, Jennifer ; Oleszek, Joyce ; Pandya, Shree ; Price, Elinora ; Yang, Michele L. ; Auerbach, Scott R.
• Description:
  Background:
  
  Duchenne muscular dystrophy is associated with progressive cardiorespiratory failure, including left ventricular dysfunction.
  
  Methods and Results:
  
  Males with probable or definite diagnosis of Duchenne muscular dystrophy, diagnosed between 1 January, 1982 and 31 December, 2011, were identified from the Muscular Dystrophy Surveillance Tracking and Research Network database. Two non-mutually exclusive groups were created: patients with ≥2 echocardiograms and non-invasive positive pressure ventilation-compliant patients with ≥1 recorded ejection fraction. Quantitative left ventricular dysfunction was defined as an ejection fraction <55%. Qualitative dysfunction was defined as mild, moderate, or severe. Progression of quantitative left ventricular dysfunction was modelled as a continuous time-varying outcome. Change in qualitative left ventricle function was assessed by the percentage of patients within each category at each age. Forty-one percent (n = 403) had ≥2 ejection fractions containing 998 qualitative assessments with a mean age at first echo of 10.8 ± 4.6 years, with an average first ejection fraction of 63.1 ± 12.6%. Mean age at first echo with an ejection fraction <55 was 15.2 ± 3.9 years. Thirty-five percent (140/403) were non-invasive positive pressure ventilation-compliant and had ejection fraction information. The estimated rate of decline in ejection fraction from first ejection fraction was 1.6% per year and initiation of non-invasive positive pressure ventilation did not change this rate.
  
  Conclusions:
  
  In our cohort, we observed that left ventricle function in patients with Duchenne muscular dystrophy declined over time, independent of non-invasive positive pressure ventilation use. Future studies are needed to examine the impact of respiratory support on cardiac function.
  
  
• Subjects:
  Adolescent Adult Article Child Child, Preschool Cohort Studies Duchenne Muscular Dystrophy Echocardiography Glucocorticoids Humans Infant Infant, Newborn Left Ventricular Dysfunction Male Muscular Dystrophy, Duchenne Non-invasive Positive Pressure Ventilation Stroke Volume Ventricular Dysfunction, Left Young Adult

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• Source:
  Cardiol Young. 30(2):171-176
• Pubmed ID:
  31964455
• Pubmed Central ID:
  PMC8941672
• Document Type:
  Journal Article
• Funding:
  U01 DD001126/DD/NCBDD CDC HHSUnited States/ ; U01 DD000187/DD/NCBDD CDC HHSUnited States/ ; U01 DD001123/DD/NCBDD CDC HHSUnited States/ ; U01DD001123/ACL/ACL HHSUnited States/ ; U01 DD000189/DD/NCBDD CDC HHSUnited States/ ; U01DD001119/ACL/ACL HHSUnited States/ ; CC999999/ImCDC/Intramural CDC HHSUnited States/ ; U01DD001126/ACL/ACL HHSUnited States/ ; U01 DD000191/DD/NCBDD CDC HHSUnited States/ ; U01 DD001119/DD/NCBDD CDC HHSUnited States/
• Volume:
  30
• Issue:
  2
• Collection(s):
  CDC Public Access
• Main Document Checksum:
  urn:sha256:9f8aa4a2eac2d9ce4f84840f0436568b7bec85e357a2da4e494f9b547df5f035
• Download URL:
  https://stacks.cdc.gov/view/cdc/115711/cdc_115711_DS1.pdf
• File Type:
  [PDF - 430.03 KB ]