Left ventricular noncompaction in Duchenne muscular dystrophy

Journal of Cardiovascular Magnetic Resonance

Kan N Hor²  D Woodrow Benson⁴  Jesse Pratt¹  Eileen King¹  Linda H Cripe²  Wojciech Mazur³  Michael D Taylor¹  Christopher J Statile¹ 

[1] Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA;Nationwide Children’s Hospital, Columbus, OH, USA;The Ohio Heart and Vascular Center at The Christ Hospital, Cincinnati, OH, USA;Children’s Hospital of Wisconsin, Milwaukee, WI, USA

关键词: Cardiac magnetic resonance imaging;    Duchenne muscular dystrophy;    Left ventricular noncompaction;   

Others  :  812381
DOI  :  10.1186/1532-429X-15-67

 received in 2013-03-14, accepted in 2013-07-07,  发布年份 2013

【 摘 要 】

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.

【 授权许可】

   
2013 Statile et al.; licensee BioMed Central Ltd.

【 图 表 】

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