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. 2020 Feb;30(2):171-176.
doi: 10.1017/S1047951119002610. Epub 2020 Jan 22.

Left ventricular dysfunction in Duchenne muscular dystrophy

Katherine A James  1 , Jane Gralla  2 , Leslie A Ridall  3 , ThuyQuynh N Do  4 , Angela S Czaja  3 , Peter M Mourani  3 , Emma Ciafaloni  5 , Christopher Cunniff  6 , Jennifer Donnelly  3 , Joyce Oleszek  3 , Shree Pandya  5 , Elinora Price  6 , Michele L Yang  3 , Scott R Auerbach  3
Affiliations

Left ventricular dysfunction in Duchenne muscular dystrophy

Katherine A James et al. Cardiol Young. 2020 Feb.

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.

Keywords: Duchenne muscular dystrophy; left ventricular dysfunction; non-invasive positive pressure ventilation.

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Conflict of interest statement

Conflicts of Interest. The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
Selection of analytic cohorts from the MD STARnet Surveillance System, 1982–2011.
Figure 2.
Figure 2.
Qualitative assessment of left ventricular dysfunction (subjective echo function), MD STARnet 1982–2011 (n = 998 echoes in 403 individuals).
See this image and copyright information in PMC

Comment in

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