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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

Distribution of positive segments by patient group. Segment 16 (apical lateral) in both DMD and LVNC most commonly fulfilled criteria for LVNC. The apical and lateral segments are more frequently positive across all groups which is consistent with previous findings in LVNC. DMD, Duchenne Muscular Dystrophy; EF, ejection fraction; LVNC, left ventricular non-compaction; NC, noncompacted length; C, compacted length.
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Figure 2: Distribution of positive segments by patient group. Segment 16 (apical lateral) in both DMD and LVNC most commonly fulfilled criteria for LVNC. The apical and lateral segments are more frequently positive across all groups which is consistent with previous findings in LVNC. DMD, Duchenne Muscular Dystrophy; EF, ejection fraction; LVNC, left ventricular non-compaction; NC, noncompacted length; C, compacted length.

Mentions: Particular LV segments that were positive for LVNC with a NC/C ratio > 2.3 were in similar locations in patients with DMD with EF > 55%, DMD with EF < 55% or LVNC. In all the subjects, the apical segments were more likely to have ratios > 2.3 with segment 16 being the most common segment to be positive. Segment 16 was positive in 73% of the primary LVNC patients, 40% of DMD subjects with an EF < 55%, and 9.1% of DMD subjects with an EF > 55%. The segments most commonly positive in the LVNC population were apical segments 13, 15 and 16. In the DMD population, it was segments 11,12 and 16. These segments correspond to the segments that have been described to have scar tissue burden with LGE analysis [24] (Figure 2).


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)

Distribution of positive segments by patient group. Segment 16 (apical lateral) in both DMD and LVNC most commonly fulfilled criteria for LVNC. The apical and lateral segments are more frequently positive across all groups which is consistent with previous findings in LVNC. DMD, Duchenne Muscular Dystrophy; EF, ejection fraction; LVNC, left ventricular non-compaction; NC, noncompacted length; C, compacted length.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 2: Distribution of positive segments by patient group. Segment 16 (apical lateral) in both DMD and LVNC most commonly fulfilled criteria for LVNC. The apical and lateral segments are more frequently positive across all groups which is consistent with previous findings in LVNC. DMD, Duchenne Muscular Dystrophy; EF, ejection fraction; LVNC, left ventricular non-compaction; NC, noncompacted length; C, compacted length.
Mentions: Particular LV segments that were positive for LVNC with a NC/C ratio > 2.3 were in similar locations in patients with DMD with EF > 55%, DMD with EF < 55% or LVNC. In all the subjects, the apical segments were more likely to have ratios > 2.3 with segment 16 being the most common segment to be positive. Segment 16 was positive in 73% of the primary LVNC patients, 40% of DMD subjects with an EF < 55%, and 9.1% of DMD subjects with an EF > 55%. The segments most commonly positive in the LVNC population were apical segments 13, 15 and 16. In the DMD population, it was segments 11,12 and 16. These segments correspond to the segments that have been described to have scar tissue burden with LGE analysis [24] (Figure 2).

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