TY - JOUR
T1 - Patterns of left ventricular remodeling in patients with Duchenne Muscular Dystrophy
T2 - A cardiac MRI study of ventricular geometry, global function, and strain
AU - Mazur, Wojciech
AU - Hor, Kan N.
AU - Germann, Joshua T.
AU - Fleck, Robert J.
AU - Al-Khalidi, Hussein R.
AU - Wansapura, Janaka P.
AU - Chung, Eugene S.
AU - Taylor, Michael D.
AU - Jefferies, John L.
AU - Benson, D. Woodrow
AU - Gottliebson, William M.
N1 - Funding Information:
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.
PY - 2012/1
Y1 - 2012/1
N2 - 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.
AB - 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.
KW - Cardiac MRI
KW - Dilated Cardiomyopathy
KW - Duchenne
KW - LVRI
KW - MVR
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U2 - 10.1007/s10554-010-9781-2
DO - 10.1007/s10554-010-9781-2
M3 - Article
C2 - 21222036
AN - SCOPUS:84861227330
SN - 1569-5794
VL - 28
SP - 99
EP - 107
JO - International Journal of Cardiovascular Imaging
JF - International Journal of Cardiovascular Imaging
IS - 1
ER -