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  • Poster presentation
  • Open Access

Left ventricular function by echocardiography correlates poorly with cardiac MRI measures in Duchenne muscular dystrophy

  • 1,
  • 1, 2,
  • 3,
  • 3,
  • 4 and
  • 1
Journal of Cardiovascular Magnetic Resonance201416 (Suppl 1) :P306

https://doi.org/10.1186/1532-429X-16-S1-P306

  • Published:

Keywords

  • Duchenne Muscular Dystrophy
  • Wall Motion Abnormality
  • Duchenne Muscular Dystrophy
  • Duchenne Muscular Dystrophy Patient
  • Annual Left Ventricular

Background

Duchenne muscular dystrophy (DMD) causes skeletal muscle weakness and cardiomyopathy (CM). Current recommendations are for annual left ventricular (LV) function assessment after age 10 years. Although echocardiographic image quality in DMD patients can be affected by scoliosis and adipose tissue, recent reviews recommend echocardiography as the standard imaging modality. We hypothesized that objective and subjective LV functional assessment by echocardiography in DMD is suboptimal compared to cardiac MRI (CMR).

Methods

Twelve DMD patients prospectively enrolled; echocardiography and CMR performed median of 0 days apart (max 22 days). Echocardiography was performed by sonographers with DMD imaging expertise. Cardiologist blinded to CMR results measured the following echocardiographic parameters: 1) M-mode fractional shortening (MMFS); 2) 2-dimensional FS (2DFS), 3) biplane LV ejection fraction (LVEF); 4) single plane LVEF; 5) 3-dimensional LVEF; 6) peak circumferential strain (εcc ); 7) subjective LVEF. CMR measures included: 1) LVEF; 2) HARP εcc analysis of tagged images; 3) Subjective segmental function. Segmental assessments by echocardiography and CMR were performed using 17-segment model. Agreement between echocardiography and CMR assessed with intraclass correlation coefficient (ICC) and Spearman correlation; subjective LVEF evaluated with weighted kappa.

Results

Mean age was 15.8 years (range 10-27). Mean LVEF by CMR was 47.4 ± 8.9%; 8 patients had CM defined as LVEF < 55% (Table 1). Subjective echocardiographic image quality rated good in 4/12 (33.3%), average 2/12 (16.7%), poor 3/12 (25%) and inadequate 3/12 (25%); none rated excellent. For echocardiography, only MMFS was measurable in all patients. Only moderate correlations were seen between MMFS and CMR LVEF (r = 0.59, p = 0.042) and echocardiographic εcc and CMR εcc (ICC = 0.52, p = 0.045). A strong correlation was seen between 2DFS and CMR LVEF (r = 0.79, p = 0.033) but 2DFS was only obtainable in 58% of patients. No significant correlations were found between other measures, including subjective LVEF (Table 2). Subjective segmental assessment was possible in 202 of 204 segments by CMR and only 137 of 204 segments by echocardiography. Of 69 segments not visualized by echocardiography, 39 had abnormal wall motion by CMR. Inferior and inferolateral walls at mid-ventricular level were most common sites of wall motion abnormalities.
Table 1

Results of Objective Measures of LV Function

Measures of LV Function

Mean ± SD

N

Echocardiographic Measures

M-mode FS

24.5 ± 6.1

12

2-Dimensional FS

27.6 ± 3.9

7

Biplane LVEF

52.1 ± 8.1

4

4 chamber LVEF

44 ± 12.2

7

3-Dimensional LVEF

36.2 ± 13.3

5

εcc

-16.5 ± 4.4

8

CMR Measures

LVEF

47.4 ± 8.9

12

εcc

-12.9 ± 3.5

12

LV (left ventricular) SD (standard deviation) FS (fractional shortening) LVEF (left ventricular ejection fraction) εcc (peak circumferential strain)

Table 2

Comparison of Echocardiography and CMR measures of LV function

Echocardiographic Measures

Adequate Echocardiographic Image Quality (N = 12)

Correlation

P-value

  

CMR LVEF

 

M-mode FS

12/12 (100%)

0.591

0.042

2-Dimensional FS

7/12 (58.3%)

0.791

0.033

Biplane LVEF

4/12 (33.3%)

-0.072

0.531

4 chamber LVEF

7/12 (58.3%)

0.52

0.09

3-Dimensional LVEF

5/12 (41.7%)

0.282

0.168

Subjective global function

12/12 (100%)

0.083

0.35

  

CMR εcc

 

εcc

8/12 (66.6%)

0.521

0.045

LVEF (left ventricular ejection fraction) FS (fractional shortening) εcc (peak circumferential strain) 1Spearman Correlation 2Intraclass Correlation Coefficient 3Weighted Kappa

Conclusions

Objective and subjective echocardiographic measures of LV function were not possible in many DMD patients and had limited correlation with CMR. Only 3 studies were rated inadequate, suggesting that, even in the face of "adequate" imaging, functional analysis by echocardiography had suboptimal correlation and unrecognized wall motion abnormalities. These discrepancies could adversely impact patient care. We recommend early consideration for CMR for annual, accurate assessment of DMD function.

Funding

The project described was supported by: 1) The American Heart Association Clinical Research Program, Grant 13CRP14530007. 2) The National Center for Research Resources, Grant UL1 RR024975-01, and is now at the National Center for Advancing Translational Sciences, Grant 2 UL1 TR000445-06. 3) The Fighting Duchenne Foundation and the Fight DMD/Jonah & Emory Discovery Grant (Nashville, TN).

Authors’ Affiliations

(1)
Department of Pediatrics, Division of Cardiology, Vanderbilt University Medical Center, Nashville, Tennessee, USA
(2)
Department of Medicine, Division of Cardiology, Vanderbilt University Medical Center, Nashville, Tennessee, USA
(3)
Biostatistics, Vanderbilt University Medical Center, Nashville, Tennessee, USA
(4)
Departments of Radiology and Radiological Sciences, Molecular Physiology and Biophysics, and Biomedical Engineering, Vanderbilt University Medical Center, Nashville, Tennessee, USA

Copyright

© Soslow et al.; licensee BioMed Central Ltd. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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