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Regional circumferential strain is a biomarker for disease severity in duchenne muscular dystrophy heart disease: a cross-sectional study.

Publication ,  Journal Article
Hor, KN; Kissoon, N; Mazur, W; Gupta, R; Ittenbach, RF; Al-Khalidi, HR; Cripe, LH; Raman, SV; Puchalski, MD; Gottliebson, WM; Benson, DW
Published in: Pediatr Cardiol
January 2015

The aim of this study is to determine the contribution of strain ε cc in mid left ventricular (LV) segments to the reduction of composite LV circumferential ε cc in assess severity of duchenne muscular dystrophy (DMD) heart disease as assessed by cardiac magnetic resonance imaging (CMR). DMD patients and control subjects were stratified by age, LV ejection fraction, and late gadolinium enhancement (LGE) status. Tagged CMR images were analyzed for global ventricular function, LGE imaging, and composite and segmental ε cc. The relationship between changes in segmental ε cc changes and LGE across patient groups was assessed by a statistical step-down model. LV ε cc exhibited segmental heterogeneity; in control subjects and young DMD patients, ε cc was greatest in LV lateral free wall segments. However, with increasing age and cardiac disease severity as demonstrated by decreased EF and development of myocardial strain the segmental differences diminished. In subjects with advanced heart disease as evidenced by reduced LV ejection fraction and presence of LGE, very little segmental heterogeneity was present. In control subjects and young DMD patients, ε cc was greatest in LV lateral free wall segments. Increased DMD heart disease severity was associated with reduced composite; ε cc diminished regional ε cc heterogeneity and positive LGE imaging. Taken together, these findings suggest that perturbation of segmental, heterogeneous ε cc is an early biomarker of disease severity in this cross-section of DMD patients.

Duke Scholars

Published In

Pediatr Cardiol

DOI

EISSN

1432-1971

Publication Date

January 2015

Volume

36

Issue

1

Start / End Page

111 / 119

Location

United States

Related Subject Headings

  • Severity of Illness Index
  • Muscular Dystrophy, Duchenne
  • Male
  • Magnetic Resonance Imaging, Cine
  • Humans
  • Gadolinium DTPA
  • Cross-Sectional Studies
  • Contrast Media
  • Child
  • Case-Control Studies
 

Citation

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MLA
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Hor, K. N., Kissoon, N., Mazur, W., Gupta, R., Ittenbach, R. F., Al-Khalidi, H. R., … Benson, D. W. (2015). Regional circumferential strain is a biomarker for disease severity in duchenne muscular dystrophy heart disease: a cross-sectional study. Pediatr Cardiol, 36(1), 111–119. https://doi.org/10.1007/s00246-014-0972-9
Hor, Kan N., Narayan Kissoon, Wojciech Mazur, Resmi Gupta, Richard F. Ittenbach, Hussein R. Al-Khalidi, Linda H. Cripe, et al. “Regional circumferential strain is a biomarker for disease severity in duchenne muscular dystrophy heart disease: a cross-sectional study.Pediatr Cardiol 36, no. 1 (January 2015): 111–19. https://doi.org/10.1007/s00246-014-0972-9.
Hor KN, Kissoon N, Mazur W, Gupta R, Ittenbach RF, Al-Khalidi HR, et al. Regional circumferential strain is a biomarker for disease severity in duchenne muscular dystrophy heart disease: a cross-sectional study. Pediatr Cardiol. 2015 Jan;36(1):111–9.
Hor, Kan N., et al. “Regional circumferential strain is a biomarker for disease severity in duchenne muscular dystrophy heart disease: a cross-sectional study.Pediatr Cardiol, vol. 36, no. 1, Jan. 2015, pp. 111–19. Pubmed, doi:10.1007/s00246-014-0972-9.
Hor KN, Kissoon N, Mazur W, Gupta R, Ittenbach RF, Al-Khalidi HR, Cripe LH, Raman SV, Puchalski MD, Gottliebson WM, Benson DW. Regional circumferential strain is a biomarker for disease severity in duchenne muscular dystrophy heart disease: a cross-sectional study. Pediatr Cardiol. 2015 Jan;36(1):111–119.
Journal cover image

Published In

Pediatr Cardiol

DOI

EISSN

1432-1971

Publication Date

January 2015

Volume

36

Issue

1

Start / End Page

111 / 119

Location

United States

Related Subject Headings

  • Severity of Illness Index
  • Muscular Dystrophy, Duchenne
  • Male
  • Magnetic Resonance Imaging, Cine
  • Humans
  • Gadolinium DTPA
  • Cross-Sectional Studies
  • Contrast Media
  • Child
  • Case-Control Studies