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Left ventricular noncompaction in Duchenne muscular dystrophy.

Statile CJ, Taylor MD, Mazur W, Cripe LH, King E, Pratt J, Benson DW, Hor KN - J Cardiovasc Magn Reson (2013)

Bottom Line: LVNC was defined as a diastolic NC/C ratio > 2.3 for any segment.Longitudinal data for 78 of the DMD boys demonstrated a mean rate of change in NC/C ratio per year of +0.36.The high prevalence of LVNC in DMD is associated with decreased LV systolic function that develops over time and may represent muscular degeneration versus compensatory remodeling.

View Article: PubMed Central - HTML - PubMed

Affiliation: Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. Christopher.statile@cchmc.org

ABSTRACT

Background: Left ventricular noncompaction (LVNC) describes deep trabeculations in the left ventricular (LV) endocardium and a thinned epicardium. LVNC is seen both as a primary cardiomyopathy and as a secondary finding in other syndromes affecting the myocardium such as neuromuscular disorders. The objective of this study is to define the prevalence of LVNC in the Duchenne Muscular Dystrophy (DMD) population and characterize its relationship to global LV function.

Methods: Cardiac magnetic resonance (CMR) was used to assess ventricular morphology and function in 151 subjects: DMD with ejection fraction (EF) > 55% (n = 66), DMD with EF < 55% (n = 30), primary LVNC (n = 15) and normal controls (n = 40). The non-compacted to compacted (NC/C) ratio was measured in each of the 16 standard myocardial segments. LVNC was defined as a diastolic NC/C ratio > 2.3 for any segment.

Results: LVNC criteria were met by 27/96 DMD patients (prevalence of 28%): 11 had an EF > 55% (prevalence of 16.7%), and 16 had an EF < 55% (prevalence of 53.3%). The median maximum NC/C ratio was 1.8 for DMD with EF > 55%, 2.46 for DMD with EF < 55%, 1.54 for the normal subjects, and 3.69 for primary LVNC patients. Longitudinal data for 78 of the DMD boys demonstrated a mean rate of change in NC/C ratio per year of +0.36.

Conclusion: The high prevalence of LVNC in DMD is associated with decreased LV systolic function that develops over time and may represent muscular degeneration versus compensatory remodeling.

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Related in: MedlinePlus

Pathologic specimen showing LVNC in a DMD patient. The image shows a piece of myocardium removed from the apex during implantation of a left ventricular assist device. There is extensive noncompacted myocardium extending form the compacted myocardium in finger-like projections. DMD, Duchenne Muscular Dystrophy.
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Figure 8: Pathologic specimen showing LVNC in a DMD patient. The image shows a piece of myocardium removed from the apex during implantation of a left ventricular assist device. There is extensive noncompacted myocardium extending form the compacted myocardium in finger-like projections. DMD, Duchenne Muscular Dystrophy.

Mentions: All three patients with available pathology were confirmed to have LVNC by standard pathologic criteria. An example myocardial wedge is shown in Figure 8. There is extensive non-compacted myocardium and relatively thin areas of compacted myocardium. The DMD patient showed extensive areas of fibrosis consistent with typical changes of DMD cardiomyopathy.


Left ventricular noncompaction in Duchenne muscular dystrophy.

Statile CJ, Taylor MD, Mazur W, Cripe LH, King E, Pratt J, Benson DW, Hor KN - J Cardiovasc Magn Reson (2013)

Pathologic specimen showing LVNC in a DMD patient. The image shows a piece of myocardium removed from the apex during implantation of a left ventricular assist device. There is extensive noncompacted myocardium extending form the compacted myocardium in finger-like projections. DMD, Duchenne Muscular Dystrophy.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3750745&req=5

Figure 8: Pathologic specimen showing LVNC in a DMD patient. The image shows a piece of myocardium removed from the apex during implantation of a left ventricular assist device. There is extensive noncompacted myocardium extending form the compacted myocardium in finger-like projections. DMD, Duchenne Muscular Dystrophy.
Mentions: All three patients with available pathology were confirmed to have LVNC by standard pathologic criteria. An example myocardial wedge is shown in Figure 8. There is extensive non-compacted myocardium and relatively thin areas of compacted myocardium. The DMD patient showed extensive areas of fibrosis consistent with typical changes of DMD cardiomyopathy.

Bottom Line: LVNC was defined as a diastolic NC/C ratio > 2.3 for any segment.Longitudinal data for 78 of the DMD boys demonstrated a mean rate of change in NC/C ratio per year of +0.36.The high prevalence of LVNC in DMD is associated with decreased LV systolic function that develops over time and may represent muscular degeneration versus compensatory remodeling.

View Article: PubMed Central - HTML - PubMed

Affiliation: Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. Christopher.statile@cchmc.org

ABSTRACT

Background: Left ventricular noncompaction (LVNC) describes deep trabeculations in the left ventricular (LV) endocardium and a thinned epicardium. LVNC is seen both as a primary cardiomyopathy and as a secondary finding in other syndromes affecting the myocardium such as neuromuscular disorders. The objective of this study is to define the prevalence of LVNC in the Duchenne Muscular Dystrophy (DMD) population and characterize its relationship to global LV function.

Methods: Cardiac magnetic resonance (CMR) was used to assess ventricular morphology and function in 151 subjects: DMD with ejection fraction (EF) > 55% (n = 66), DMD with EF < 55% (n = 30), primary LVNC (n = 15) and normal controls (n = 40). The non-compacted to compacted (NC/C) ratio was measured in each of the 16 standard myocardial segments. LVNC was defined as a diastolic NC/C ratio > 2.3 for any segment.

Results: LVNC criteria were met by 27/96 DMD patients (prevalence of 28%): 11 had an EF > 55% (prevalence of 16.7%), and 16 had an EF < 55% (prevalence of 53.3%). The median maximum NC/C ratio was 1.8 for DMD with EF > 55%, 2.46 for DMD with EF < 55%, 1.54 for the normal subjects, and 3.69 for primary LVNC patients. Longitudinal data for 78 of the DMD boys demonstrated a mean rate of change in NC/C ratio per year of +0.36.

Conclusion: The high prevalence of LVNC in DMD is associated with decreased LV systolic function that develops over time and may represent muscular degeneration versus compensatory remodeling.

Show MeSH
Related in: MedlinePlus