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Patterns of left ventricular remodeling in patients with Duchenne Muscular Dystrophy: a cardiac MRI study of ventricular geometry, global function, and strain

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Abstract

The cardiac disease ubiquitously associated in Duchenne Muscular Dystrophy (DMD) has traditionally been considered a progressive dilated cardiomyopathy (DCM). However, left ventricular (LV) dilatation as measured with cardiac MRI has not been a consistent finding in this population, even as circumferential strain (εcc) declines with advancing disease. We hypothesized that a distinct pattern of changes in LV geometry, during the course of εcc decline, distinguishes DMD associated heart disease from DCM. Using CMR, LV end-diastolic volume (EDV), mass (LVM), ejection fraction, εcc and myocardial delayed enhancement (MDE) were determined in DMD patients and normal control subjects. The LV Remodeling Index (LVRI) was calculated as the ratio of LV Mass to Volume (LVM/EDV). Statistical comparisons between all LV parameters and genotype were also performed. Median LVRI in DMD (n = 127) and control subjects (n = 41) were different (0.75 vs. 0.65, P = 0.0150) but within normal range. Furthermore, the median LVRI in DMD boys with reduced LV systolic function was significantly reduced compared to those with normal LV systolic function (0.64 vs. 0.75, P = 0.0974). However, the presence of MDE was associated with a lower median LVRI (0.57 vs. 0.76, P = 0.0471). Regression analysis showed no significant correlation between εcc and LVRI (r = −0.03). The LVRI of DMD patients is unexpectedly normal and not correlated with εcc. Based on these findings, DMD-associated heart disease exhibits a unique remodeling pattern distinct from DCM.

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Acknowledgments

We wish to recognize additional members of the Cardiac MRI and DMD Clinical Care Teams at CCHMC (Linda Cripe MD, Robert Spicer MD, Kathi Kinnett MSN CNP, Sean Hagenbuch MD, Eric Crotty MD, Kathy Helton MD Daniel Podberesky MD, and Amy Tipton, BFA) for clinical and CMR data acquisition and analysis. Supported by grants from the Children’s Heart Association of Cincinnati (WMG), Parent Project Muscular Dystrophy (NK), and HL069712 (DWB) from the NIH.

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Authors and Affiliations

  1. The Heart and Vascular Center at the Christ Hospital, Cincinnati, OH, USA

    Wojciech Mazur, Eugene S. Chung & John L. Jefferies

  2. The Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

    Kan N. Hor, Joshua T. Germann, Michael D. Taylor, John L. Jefferies, D. Woodrow Benson & William M. Gottliebson

  3. The Department of Radiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

    Robert J. Fleck & Janaka P. Wansapura

  4. Duke University School of Medicine, Durham, NC, USA

    Hussein R. Al-Khalidi

Authors
  1. Wojciech Mazur
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  2. Kan N. Hor
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  3. Joshua T. Germann
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  4. Robert J. Fleck
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  5. Hussein R. Al-Khalidi
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  6. Janaka P. Wansapura
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  7. Eugene S. Chung
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  8. Michael D. Taylor
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  9. John L. Jefferies
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  10. D. Woodrow Benson
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  11. William M. Gottliebson
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Correspondence to Wojciech Mazur.

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Dedicated to memory of William M. Gottliebson.

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Mazur, W., Hor, K.N., Germann, J.T. et al. Patterns of left ventricular remodeling in patients with Duchenne Muscular Dystrophy: a cardiac MRI study of ventricular geometry, global function, and strain. Int J Cardiovasc Imaging 28, 99–107 (2012). https://doi.org/10.1007/s10554-010-9781-2

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  • DOI: https://doi.org/10.1007/s10554-010-9781-2

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