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Increased susceptibility of the left lateral free wall to myocardial delayed enhancement in Duchenne Muscular Dystrophy: progressive systolic dysfunction demonstrable by CMR regional strain analysis

Background

Cardiac magnetic resonance imaging (CMR) has demonstrated reductions in peak LV myocardial circumferential strain (εcc) despite normal ejection fraction (EF) in Duchenne Muscular Dystrophy (DMD) patients. We hypothesized that the increased initial contractility of the lateral LV free wall makes that region more susceptible to myocardial injury with subsequent fibrosis than the septum in DMD patients.

Methods

We analyzed regional εcc from myocardial tagged CMR images on the mid-papillary level LV slice (using HARP™ software) from 14 DMD males with global cardiac dysfunction (LV EF < 55%) and myocardial delayed enhancement (MDE, a marker of myocardial fibrosis), as well as from 13 age-matched control males with normal cardiac function. Regions were assigned based on standard coronary perfusion regions. Regional Δεcc was computed as the difference between normal and DMD subject εcc per region.

Results

In controls, the lateral free wall regions had a greater baseline εcc than the septal regions. In DMD patients, Δεcc was consistently greater in magnitude in the lateral free wall regions when compared to the Δεcc of the septal regions (Figure 1). MDE was consistently detected in these same lateral free wall regions.

Figure 1
figure1

Regional distribution of MDE and corresponding Δε cc .

Conclusion

Changes in εcc show that the regions with greatest contractility in control subjects (the lateral free wall) are the most susceptible to injury in DMD patients, as exemplified both by the greatest reduction in regional εcc and the development of MDE in those regions.

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Correspondence to Narayan Kissoon.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (https://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Kissoon, N., Hor, K.N., Wansapura, J.P. et al. Increased susceptibility of the left lateral free wall to myocardial delayed enhancement in Duchenne Muscular Dystrophy: progressive systolic dysfunction demonstrable by CMR regional strain analysis. J Cardiovasc Magn Reson 11, P12 (2009). https://doi.org/10.1186/1532-429X-11-S1-P12

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Keywords

  • Cardiac Magnetic Resonance
  • Duchenne Muscular Dystrophy
  • Duchenne Muscular Dystrophy
  • Duchenne Muscular Dystrophy Patient
  • Normal Ejection Fraction