Left ventricular dysfunction in Duchenne muscular dystrophy

Katherine A. James, Jane Gralla, Leslie A. Ridall, Thuy Quynh N. Do, Angela S. Czaja, Peter M. Mourani, Emma Ciafaloni, Christopher Cunniff, Jennifer Donnelly, Joyce Oleszek, Shree Pandya, Elinora Price, Michele L. Yang, Scott R. Auerbach

Research output: Contribution to journalArticlepeer-review

3 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)171-176
Number of pages6
JournalCardiology in the Young
Volume30
Issue number2
DOIs
StatePublished - 2020

Keywords

  • Duchenne muscular dystrophy
  • left ventricular dysfunction
  • non-invasive positive pressure ventilation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Cardiology and Cardiovascular Medicine

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