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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.

Publication ,  Journal Article
Mazur, W; Hor, KN; Germann, JT; Fleck, RJ; Al-Khalidi, HR; Wansapura, JP; Chung, ES; Taylor, MD; Jefferies, JL; Benson, DW; Gottliebson, WM
Published in: Int J Cardiovasc Imaging
January 2012

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 (ε(cc)) declines with advancing disease. We hypothesized that a distinct pattern of changes in LV geometry, during the course of ε(cc) decline, distinguishes DMD associated heart disease from DCM. Using CMR, LV end-diastolic volume (EDV), mass (LVM), ejection fraction, ε(cc) 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 ε(cc) and LVRI (r = -0.03). The LVRI of DMD patients is unexpectedly normal and not correlated with ε(cc.) Based on these findings, DMD-associated heart disease exhibits a unique remodeling pattern distinct from DCM.

Duke Scholars

Published In

Int J Cardiovasc Imaging

DOI

EISSN

1875-8312

Publication Date

January 2012

Volume

28

Issue

1

Start / End Page

99 / 107

Location

United States

Related Subject Headings

  • Young Adult
  • Ventricular Remodeling
  • Ventricular Function, Left
  • Statistics, Nonparametric
  • Nuclear Medicine & Medical Imaging
  • Muscular Dystrophy, Duchenne
  • Male
  • Magnetic Resonance Imaging, Cine
  • Image Enhancement
  • Humans
 

Citation

APA
Chicago
ICMJE
MLA
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Mazur, W., Hor, K. N., Germann, J. T., Fleck, R. J., Al-Khalidi, H. R., Wansapura, J. P., … Gottliebson, W. M. (2012). 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(1), 99–107. https://doi.org/10.1007/s10554-010-9781-2
Mazur, Wojciech, Kan N. Hor, Joshua T. Germann, Robert J. Fleck, Hussein R. Al-Khalidi, Janaka P. Wansapura, Eugene S. Chung, 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, no. 1 (January 2012): 99–107. https://doi.org/10.1007/s10554-010-9781-2.
Mazur W, Hor KN, Germann JT, Fleck RJ, Al-Khalidi HR, Wansapura JP, 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. 2012 Jan;28(1):99–107.
Mazur, Wojciech, 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, vol. 28, no. 1, Jan. 2012, pp. 99–107. Pubmed, doi:10.1007/s10554-010-9781-2.
Mazur W, Hor KN, Germann JT, Fleck RJ, Al-Khalidi HR, Wansapura JP, Chung ES, Taylor MD, Jefferies JL, Benson DW, Gottliebson WM. 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. 2012 Jan;28(1):99–107.

Published In

Int J Cardiovasc Imaging

DOI

EISSN

1875-8312

Publication Date

January 2012

Volume

28

Issue

1

Start / End Page

99 / 107

Location

United States

Related Subject Headings

  • Young Adult
  • Ventricular Remodeling
  • Ventricular Function, Left
  • Statistics, Nonparametric
  • Nuclear Medicine & Medical Imaging
  • Muscular Dystrophy, Duchenne
  • Male
  • Magnetic Resonance Imaging, Cine
  • Image Enhancement
  • Humans