PMID- 25085262
OWN - NLM
STAT- MEDLINE
DCOM- 20150716
LR  - 20211021
IS  - 1432-1971 (Electronic)
IS  - 0172-0643 (Linking)
VI  - 36
IP  - 1
DP  - 2015 Jan
TI  - Regional circumferential strain is a biomarker for disease severity in duchenne 
      muscular dystrophy heart disease: a cross-sectional study.
PG  - 111-9
LID - 10.1007/s00246-014-0972-9 [doi]
AB  - The aim of this study is to determine the contribution of strain epsilon cc in mid left 
      ventricular (LV) segments to the reduction of composite LV circumferential epsilon 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 epsilon cc. The relationship between changes 
      in segmental epsilon cc changes and LGE across patient groups was assessed by a 
      statistical step-down model. LV epsilon cc exhibited segmental heterogeneity; in 
      control subjects and young DMD patients, epsilon 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, epsilon cc was 
      greatest in LV lateral free wall segments. Increased DMD heart disease severity 
      was associated with reduced composite; epsilon cc diminished regional epsilon cc 
      heterogeneity and positive LGE imaging. Taken together, these findings suggest 
      that perturbation of segmental, heterogeneous epsilon cc is an early biomarker of 
      disease severity in this cross-section of DMD patients.
FAU - Hor, Kan N
AU  - Hor KN
AD  - Nationwide Children's Hospital, Columbus, OH, USA.
FAU - Kissoon, Narayan
AU  - Kissoon N
FAU - Mazur, Wojciech
AU  - Mazur W
FAU - Gupta, Resmi
AU  - Gupta R
FAU - Ittenbach, Richard F
AU  - Ittenbach RF
FAU - Al-Khalidi, Hussein R
AU  - Al-Khalidi HR
FAU - Cripe, Linda H
AU  - Cripe LH
FAU - Raman, Subha V
AU  - Raman SV
FAU - Puchalski, Michael D
AU  - Puchalski MD
FAU - Gottliebson, William M
AU  - Gottliebson WM
FAU - Benson, D Woodrow
AU  - Benson DW
LA  - eng
PT  - Journal Article
DEP - 20140802
PL  - United States
TA  - Pediatr Cardiol
JT  - Pediatric cardiology
JID - 8003849
RN  - 0 (Biomarkers)
RN  - 0 (Contrast Media)
RN  - K2I13DR72L (Gadolinium DTPA)
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Biomarkers
MH  - Cardiomyopathies/*etiology/*physiopathology
MH  - Case-Control Studies
MH  - Child
MH  - Contrast Media
MH  - Cross-Sectional Studies
MH  - Gadolinium DTPA
MH  - Humans
MH  - Magnetic Resonance Imaging, Cine
MH  - Male
MH  - Muscular Dystrophy, Duchenne/*complications
MH  - Severity of Illness Index
EDAT- 2014/08/03 06:00
MHDA- 2015/07/17 06:00
CRDT- 2014/08/03 06:00
PHST- 2014/03/13 00:00 [received]
PHST- 2014/07/16 00:00 [accepted]
PHST- 2014/08/03 06:00 [entrez]
PHST- 2014/08/03 06:00 [pubmed]
PHST- 2015/07/17 06:00 [medline]
AID - 10.1007/s00246-014-0972-9 [doi]
PST - ppublish
SO  - Pediatr Cardiol. 2015 Jan;36(1):111-9. doi: 10.1007/s00246-014-0972-9. Epub 2014 
      Aug 2.