title: Patterns of left ventricular remodeling in patients with Duchenne Muscular Dystrophy: a cardiac MRI study of ventricular geometry, global function, and strain.
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.
authors.affiliation: The Heart and Vascular Center at the Christ Hospital, Cincinnati, OH, USA. mazurw@ohioheart.org
authors.forename: Wojciech
authors.initials: W
authors.lastname: Mazur
authors.forename: Kan N
authors.initials: KN
authors.lastname: Hor
authors.forename: Joshua T
authors.initials: JT
authors.lastname: Germann
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Fleck
authors.forename: Hussein R
authors.initials: HR
authors.lastname: Al-Khalidi
authors.forename: Janaka P
authors.initials: JP
authors.lastname: Wansapura
authors.forename: Eugene S
authors.initials: ES
authors.lastname: Chung
authors.forename: Michael D
authors.initials: MD
authors.lastname: Taylor
authors.forename: John L
authors.initials: JL
authors.lastname: Jefferies
authors.forename: D Woodrow
authors.initials: DW
authors.lastname: Benson
authors.forename: William M
authors.initials: WM
authors.lastname: Gottliebson
mesh_terms: D000293:Adolescent; D000328:Adult; D000704:Analysis of Variance; D002648:Child; D002675:Child, Preschool; D003287:Contrast Media; D019786:Gadolinium DTPA; D006352:Heart Ventricles / Q000473:pathology* / Q000503:physiopathology; D006801:Humans; D007089:Image Enhancement / Q000379:methods; D019028:Magnetic Resonance Imaging, Cine / Q000379:methods*; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000473:pathology* / Q000503:physiopathology; D018709:Statistics, Nonparametric; D016277:Ventricular Function, Left; D020257:Ventricular Remodeling; D055815:Young Adult
chemical_list: D003287:Contrast Media; D019786:Gadolinium DTPA