Journal
INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING
Volume 28, Issue 1, Pages 99-107Publisher
SPRINGER
DOI: 10.1007/s10554-010-9781-2
Keywords
Duchenne; Cardiac MRI; Dilated Cardiomyopathy; LVRI; MVR
Funding
- Children's Heart Association of Cincinnati
- Parent Project Muscular Dystrophy
- NIH [HL069712]
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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 (epsilon(cc)) declines with advancing disease. We hypothesized that a distinct pattern of changes in LV geometry, during the course of epsilon(cc) decline, distinguishes DMD associated heart disease from DCM. Using CMR, LV end-diastolic volume (EDV), mass (LVM), ejection fraction, epsilon(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 epsilon(cc) and LVRI (r = -0.03). The LVRI of DMD patients is unexpectedly normal and not correlated with epsilon(cc.) Based on these findings, DMD-associated heart disease exhibits a unique remodeling pattern distinct from DCM.
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