Patterns of left ventricular remodeling in patients with Duchenne Muscular Dystrophy

A cardiac MRI study of ventricular geometry, global function, and strain

Wojciech Mazur, Kan N. Hor, Joshua T. Germann, Robert J. Fleck, Hussein R. Al-Khalidi, Janaka P. Wansapura, Eugene S. Chung, Michael D. Taylor, John Jefferies, D. Woodrow Benson, William M. Gottliebson

Research output: Contribution to journalArticle

25 Citations (Scopus)

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 (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.

Original languageEnglish (US)
Pages (from-to)99-107
Number of pages9
JournalInternational Journal of Cardiovascular Imaging
Volume28
Issue number1
DOIs
StatePublished - Jan 1 2012
Externally publishedYes

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Ventricular Remodeling
Duchenne Muscular Dystrophy
Dilated Cardiomyopathy
Heart Diseases
Left Ventricular Function
Stroke Volume
Dilatation
Reference Values
Genotype
Regression Analysis
Population

All Science Journal Classification (ASJC) codes

  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

Cite this

Patterns of left ventricular remodeling in patients with Duchenne Muscular Dystrophy : A cardiac MRI study of ventricular geometry, global function, and strain. / Mazur, Wojciech; Hor, Kan N.; Germann, Joshua T.; Fleck, Robert J.; Al-Khalidi, Hussein R.; Wansapura, Janaka P.; Chung, Eugene S.; Taylor, Michael D.; Jefferies, John; Benson, D. Woodrow; Gottliebson, William M.

In: International Journal of Cardiovascular Imaging, Vol. 28, No. 1, 01.01.2012, p. 99-107.

Research output: Contribution to journalArticle

Mazur, Wojciech ; Hor, Kan N. ; Germann, Joshua T. ; Fleck, Robert J. ; Al-Khalidi, Hussein R. ; Wansapura, Janaka P. ; Chung, Eugene S. ; Taylor, Michael D. ; Jefferies, John ; Benson, D. Woodrow ; Gottliebson, William M. / Patterns of left ventricular remodeling in patients with Duchenne Muscular Dystrophy : A cardiac MRI study of ventricular geometry, global function, and strain. In: International Journal of Cardiovascular Imaging. 2012 ; Vol. 28, No. 1. pp. 99-107.
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AU - Fleck, Robert J.

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AU - Wansapura, Janaka P.

AU - Chung, Eugene S.

AU - Taylor, Michael D.

AU - Jefferies, John

AU - Benson, D. Woodrow

AU - Gottliebson, William M.

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