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
Date
01/12/2011Pubmed ID
21222036DOI
10.1007/s10554-010-9781-2Scopus ID
2-s2.0-84861227330 (requires institutional sign-in at Scopus site) 37 CitationsAbstract
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.
Author List
Mazur W, Hor KN, Germann JT, Fleck RJ, Al-Khalidi HR, Wansapura JP, Chung ES, Taylor MD, Jefferies JL, Benson DW, Gottliebson WMMESH terms used to index this publication - Major topics in bold
AdolescentAdult
Analysis of Variance
Child
Child, Preschool
Contrast Media
Gadolinium DTPA
Heart Ventricles
Humans
Image Enhancement
Magnetic Resonance Imaging, Cine
Male
Muscular Dystrophy, Duchenne
Statistics, Nonparametric
Ventricular Function, Left
Ventricular Remodeling
Young Adult