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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

Example of measurement of noncompacted: compacted ratio in the short axis view of CMR of a DMD patient.
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Figure 1: Example of measurement of noncompacted: compacted ratio in the short axis view of CMR of a DMD patient.

Mentions: The LVNC status was assessed according to a standard metric previously described [14]. Using a high fidelity digital ruler within AMICAS PACS, the non-compacted to compacted ratio (NC/C) was measured in all 16 segments according to the AHA 16 segment model. The apical cap was not included in the assessment. All measurements were made by the primary investigator. The myocardium was considered to be positive for LVNC if the noncompacted to compacted ratio was greater than 2.3:1 in diastole in one segment as described by Petersen et al. [14]. A similar technique was used by Dawson et al. in a study describing the characteristics of compacted and non-compacted ratios [22] (Figure 1). The maximal NC/C ratio was defined as the largest NC/C in any segment. Care was taken to avoid areas of multihead attachment of the papillary muscles to the left ventricle [23].


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)

Example of measurement of noncompacted: compacted ratio in the short axis view of CMR of a DMD patient.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: Example of measurement of noncompacted: compacted ratio in the short axis view of CMR of a DMD patient.
Mentions: The LVNC status was assessed according to a standard metric previously described [14]. Using a high fidelity digital ruler within AMICAS PACS, the non-compacted to compacted ratio (NC/C) was measured in all 16 segments according to the AHA 16 segment model. The apical cap was not included in the assessment. All measurements were made by the primary investigator. The myocardium was considered to be positive for LVNC if the noncompacted to compacted ratio was greater than 2.3:1 in diastole in one segment as described by Petersen et al. [14]. A similar technique was used by Dawson et al. in a study describing the characteristics of compacted and non-compacted ratios [22] (Figure 1). The maximal NC/C ratio was defined as the largest NC/C in any segment. Care was taken to avoid areas of multihead attachment of the papillary muscles to the left ventricle [23].

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