TY - JOUR
T1 - Detection of Progressive Cardiac Dysfunction by Serial Evaluation of Circumferential Strain in Patients With Duchenne Muscular Dystrophy
AU - Hagenbuch, Sean C.
AU - Gottliebson, William M.
AU - Wansapura, Janaka
AU - Mazur, Wojciech
AU - Fleck, Robert
AU - Benson, D. Woodrow
AU - Hor, Kan N.
N1 - Funding Information:
This study was supported in part by the Children's Heart Association of Cincinnati (to W. M. Gottliebson) and grant HL069712 from the National Institutes of Health , Bethesda, Maryland (to D. W. Benson).
Copyright:
Copyright 2010 Elsevier B.V., All rights reserved.
PY - 2010/5/15
Y1 - 2010/5/15
N2 - The present study evaluated progressive cardiac dysfunction using serial circumferential strain (εcc) measurements in patients with Duchenne muscular dystrophy (DMD). DMD is characterized by progressive cardiac dysfunction and myocardial fibrosis late in the disease process. We hypothesized that serial εcc changes could be detected in individual patients with DMD during a time when the left ventricular ejection fraction (EF) changes are insignificant. Cardiac magnetic resonance imaging data from patients with DMD were evaluated. The left ventricular EF was calculated from steady-state free precession cine images and the composite εcc measurement from tagged cine images. The serial εcc and EF values for each patient were analyzed using the Wilcoxon sign rank test. Data from 51 patients with DMD (2 studies per patient, mean age at the initial study 11.8 ± 3.5 years, range 7.4 to 25.4) were analyzed, with a mean interval between cardiac magnetic resonance studies of 15.6 ± 6.0 months (range 6.2 to 28.1). In the interval between studies, the εcc had decreased in all patients with DMD. The average decrease was 1.8 ± 1.3 (p <0.001). However, the EF had decreased in 33 of the 51 patients and had increased in 18 of the 51 patients. On average, the EF decreased by 2.9 ± 8.57% (p = NS). In conclusion, in patients with DMD, εcc abnormalities indicate progression within a relatively short period when the EF changes were not significant. Serial εcc measurements might provide reliable monitoring of the progression of DMD-associated cardiac dysfunction before overt heart failure develops, because it is more sensitive than the EF.
AB - The present study evaluated progressive cardiac dysfunction using serial circumferential strain (εcc) measurements in patients with Duchenne muscular dystrophy (DMD). DMD is characterized by progressive cardiac dysfunction and myocardial fibrosis late in the disease process. We hypothesized that serial εcc changes could be detected in individual patients with DMD during a time when the left ventricular ejection fraction (EF) changes are insignificant. Cardiac magnetic resonance imaging data from patients with DMD were evaluated. The left ventricular EF was calculated from steady-state free precession cine images and the composite εcc measurement from tagged cine images. The serial εcc and EF values for each patient were analyzed using the Wilcoxon sign rank test. Data from 51 patients with DMD (2 studies per patient, mean age at the initial study 11.8 ± 3.5 years, range 7.4 to 25.4) were analyzed, with a mean interval between cardiac magnetic resonance studies of 15.6 ± 6.0 months (range 6.2 to 28.1). In the interval between studies, the εcc had decreased in all patients with DMD. The average decrease was 1.8 ± 1.3 (p <0.001). However, the EF had decreased in 33 of the 51 patients and had increased in 18 of the 51 patients. On average, the EF decreased by 2.9 ± 8.57% (p = NS). In conclusion, in patients with DMD, εcc abnormalities indicate progression within a relatively short period when the EF changes were not significant. Serial εcc measurements might provide reliable monitoring of the progression of DMD-associated cardiac dysfunction before overt heart failure develops, because it is more sensitive than the EF.
UR - http://www.scopus.com/inward/record.url?scp=77951667335&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77951667335&partnerID=8YFLogxK
U2 - 10.1016/j.amjcard.2009.12.070
DO - 10.1016/j.amjcard.2009.12.070
M3 - Article
C2 - 20451693
AN - SCOPUS:77951667335
SN - 0002-9149
VL - 105
SP - 1451
EP - 1455
JO - American Journal of Cardiology
JF - American Journal of Cardiology
IS - 10
ER -