Detection of progressive cardiac dysfunction by serial evaluation of circumferential strain in patients with Duchenne muscular dystrophy. Am J Cardiol 2010 May 15;105(10):1451-5
Date
05/11/2010Pubmed ID
20451693DOI
10.1016/j.amjcard.2009.12.070Scopus ID
2-s2.0-77951667335 (requires institutional sign-in at Scopus site) 63 CitationsAbstract
The present study evaluated progressive cardiac dysfunction using serial circumferential strain (epsilon(cc)) 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 epsilon(cc) 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 epsilon(cc) measurement from tagged cine images. The serial epsilon(cc) 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 epsilon(cc) 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, epsilon(cc) abnormalities indicate progression within a relatively short period when the EF changes were not significant. Serial epsilon(cc) 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.
Author List
Hagenbuch SC, Gottliebson WM, Wansapura J, Mazur W, Fleck R, Benson DW, Hor KNMESH terms used to index this publication - Major topics in bold
AdolescentAge Factors
Cardiomyopathies
Child
Cohort Studies
Disease Progression
Female
Fibrosis
Humans
Magnetic Resonance Imaging
Male
Monitoring, Physiologic
Muscular Dystrophy, Duchenne
Probability
Prognosis
Risk Assessment
Severity of Illness Index
Sex Factors
Statistics, Nonparametric
Stroke Volume
Survival Rate
Time Factors
Ventricular Dysfunction, Left
