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01-01-2015 | Original Article

Regional Circumferential Strain is a Biomarker for Disease Severity in Duchenne Muscular Dystrophy Heart Disease: A Cross-Sectional Study

Authors: Kan N. Hor, Narayan Kissoon, Wojciech Mazur, Resmi Gupta, Richard F. Ittenbach, Hussein R. Al-Khalidi, Linda H. Cripe, Subha V. Raman, Michael D. Puchalski, William M. Gottliebson, D. Woodrow Benson

Published in: Pediatric Cardiology | Issue 1/2015

Abstract

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.

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Title: Regional Circumferential Strain is a Biomarker for Disease Severity in Duchenne Muscular Dystrophy Heart Disease: A Cross-Sectional Study
Authors: Kan N. Hor
Narayan Kissoon
Wojciech Mazur
Resmi Gupta
Richard F. Ittenbach
Hussein R. Al-Khalidi
Linda H. Cripe
Subha V. Raman
Michael D. Puchalski
William M. Gottliebson
D. Woodrow Benson
Publication date: 01-01-2015
Publisher: Springer US
Published in: Pediatric Cardiology / Issue 1/2015
Print ISSN: 0172-0643
Electronic ISSN: 1432-1971
DOI: https://doi.org/10.1007/s00246-014-0972-9

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Regional Circumferential Strain is a Biomarker for Disease Severity in Duchenne Muscular Dystrophy Heart Disease: A Cross-Sectional Study

Abstract

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Springer Medicine

Abstract

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