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Comparative Study
. 2009 Apr 7;53(14):1204-10.
doi: 10.1016/j.jacc.2008.12.032.

Circumferential strain analysis identifies strata of cardiomyopathy in Duchenne muscular dystrophy: a cardiac magnetic resonance tagging study

Kan N Hor  1 , Janaka Wansapura, Larry W Markham, Wojciech Mazur, Linda H Cripe, Robert Fleck, D Woodrow Benson, William M Gottliebson
Affiliations
Comparative Study

Circumferential strain analysis identifies strata of cardiomyopathy in Duchenne muscular dystrophy: a cardiac magnetic resonance tagging study

Kan N Hor et al. J Am Coll Cardiol. .

Abstract

Objectives: This study sought to evaluate the natural history of occult cardiac dysfunction in Duchenne muscular dystrophy (DMD).

Background: Duchenne muscular dystrophy is characterized by progressive cardiac dysfunction and myocardial fibrosis late in the disease process. We hypothesized that left ventricular myocardial peak circumferential strain (epsilon(cc)) would decrease in DMD before global systolic functional abnormalities regardless of age or ventricular ejection fraction (EF).

Methods: We evaluated cardiac magnetic resonance image (MRI) data from 70 DMD patients and 16 aged-matched control subjects. Standard imaging data included steady-state free precession short-axis cine stack images, cine myocardial tagged images, and myocardial delayed enhancement (MDE) (an indicator of myocardial fibrosis) sequences. Analysis was performed with QMASS (Medis Medical Imaging Systems, Leiden, the Netherlands) and HARP (Diagnosoft, Palo Alto, California) software. The DMD patient data were subdivided by age (<10 or >10 years), EF (>55% or <55%), and the presence or absence of MDE.

Results: The DMD patients with normal EF had reduced epsilon(cc) at an early age (<10 years) compared with control subjects (p < 0.01). The DMD patients age >10 years with normal EF had further decline in epsilon(cc) compared with younger DMD patients (p < 0.01). There was further decline in epsilon(cc) with age in patients with reduced EF (p < 0.01) without MDE. The oldest patients, with both reduced EF and positive MDE, exhibited the lowest epsilon(cc). None of the patients had ventricular hypertrophy.

Conclusions: Myocardial strain abnormalities are prevalent in young DMD patients despite normal EF, and these strain values continue to decline with advancing age. Strain analysis in combination with standard MRI and MDE imaging provides a means to stratify DMD cardiomyopathy.

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Conflict of interest statement

Conflict of Interest and Financial Disclosure: None

Figures

Figure 1
Figure 1. Group stratification
Stratification of DMD patients is based on age, ejection fraction (EF) and presence or absence of delayed enhancement (MDE).
Figure 2
Figure 2. CMR cardiac image
The short axis of the mid-ventricle is obtained from the four-chamber view at the level of the papillary muscles with a tag sequence. Mesh overlaying of the tag image using a harmonic phase (HARP) software (Diagnosoft Inc.). Both the four-chamber and the tag images are shown during early systole.
Figure 3
Figure 3. Delayed enhancement (MDE), a CMR marker of myocardial fibrosis
MDE in the short axis (A) and long axis (B) planes indicates myocardial fibrosis in a 20 year old DMD patient as shown by the white arrows.
Figure 4
Figure 4. Box plot of ejection fraction (EF) per strata
Normal EF is seen in controls (Group A) and also in DMD patients (Groups B and C). Progressive decline in EF is seen in older patients (Group D), with further decline once MDE is present (Group E).
Figure 5
Figure 5. Graphs of εcc values per strata
(A). Bar graph shows statistically significant (p< 0.05) progressive reduction in εcc for each strata (Groups B–E) compared to controls (Group A). In addition, each strata is statistically different from other strata (B vs C, C vs D, D vs E). (B). Scatter graph of εcc of control (A) and DMD patients (B–E). No control subjects have εcc < −16% and no DMD subjects have εcc > − 16%. The asterisk (*) indicates the mean εcc of each group.
See this image and copyright information in PMC

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