Limits...
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.

Show MeSH

Related in: MedlinePlus

Noncompacted length vs time. This graph shows the progression of noncompacted length over time in all DMD patients that longitudinal data was available. There is an increase in noncompacted length over time. DMD, Duchenne Muscular Dystrophy; NC, noncompacted length.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC3750745&req=5

Figure 5: Noncompacted length vs time. This graph shows the progression of noncompacted length over time in all DMD patients that longitudinal data was available. There is an increase in noncompacted length over time. DMD, Duchenne Muscular Dystrophy; NC, noncompacted length.

Mentions: There was longitudinal data available for 78 of the DMD boys. The median rate of change in NC length per year was +0.92 mm (IQR 0.34, 2.04). The median rate of change in C length was −0.36 mm (IQR 0.78, 0). The median rate of change in NC/C ratio per year was +0.36 (IQR 0.14, 0.68). In DMD patients with serial data available, the noncompacted layer increased in thickness as the compacted layer decreased in thickness, leading to an increase in NC/C ratio (Figures 5, 6, and 7).


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)

Noncompacted length vs time. This graph shows the progression of noncompacted length over time in all DMD patients that longitudinal data was available. There is an increase in noncompacted length over time. DMD, Duchenne Muscular Dystrophy; NC, noncompacted length.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 5: Noncompacted length vs time. This graph shows the progression of noncompacted length over time in all DMD patients that longitudinal data was available. There is an increase in noncompacted length over time. DMD, Duchenne Muscular Dystrophy; NC, noncompacted length.
Mentions: There was longitudinal data available for 78 of the DMD boys. The median rate of change in NC length per year was +0.92 mm (IQR 0.34, 2.04). The median rate of change in C length was −0.36 mm (IQR 0.78, 0). The median rate of change in NC/C ratio per year was +0.36 (IQR 0.14, 0.68). In DMD patients with serial data available, the noncompacted layer increased in thickness as the compacted layer decreased in thickness, leading to an increase in NC/C ratio (Figures 5, 6, and 7).

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