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Myocardial Strain Using Cardiac MR Feature Tracking and Speckle Tracking Echocardiography in Duchenne Muscular Dystrophy Patients

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Abstract

Duchenne muscular dystrophy (DMD) is an inherited X-linked disorder with an incidence of 1 in 3500 male births, and cardiomyopathy is becoming the leading cause of death. While Cardiac MRI (CMR) and late gadolinium enhancement (LGE) are important tools in recognizing myocardial involvement, myocardial strain imaging may demonstrate early changes and allow patients to avoid gadolinium contrast. We performed CMR feature tracking (FT) and echo-based speckle tracking (STE) strain measures on DMD patients and age/sex matched controls who had received a CMR with contrast and transthoracic echocardiogram. Data were collected for longitudinal strain in the apical four-chamber view and circumferential strain in the mid-papillary parasternal short axis. Segmental wall analysis was performed and compared with the presence of LGE. Data were analyzed using student’s t tests or one-way ANOVA adjusting for multiple comparisons. We measured 24 subjects with DMD and 8 controls. Thirteen of 24 DMD subjects were LGE positive only in the lateral segments in short-axis views. Average circumferential strain (CS) measured by FT was significantly decreased in DMD compared to controls (− 18.8 ± 6.1 vs. − 25.5 ± 3.2; p < 0.001) and showed significant differences in the anterolateral, inferolateral, and inferior segments. Average CS by STE trended towards significance (p = 0.06) but showed significance in only the inferior segment. FT showed significant differences in the inferolateral segment between LGE positive (− 15.5 ± 9.0) and LGE negative (− 18.2 ± 8.3) in DMD subjects compared to controls (− 28.6 ± 7.3; p ≤ 0.04). FT also showed significant differences between anteroseptal and inferolateral segments within LGE-positive (p < 0.003) and LGE-negative (p < 0.03) DMD subjects while STE did not. There were no significant differences in longitudinal strain measures. CMR-FT-derived myocardial strain was able to demonstrate differences between subjects with DMD and controls not detected by STE. FT was also able to demonstrate differences in LGE-positive and LGE-negative segments within patients with DMD. FT may be able to predict LGE-positive segments in DMD without the use of gadolinium contrast.

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Funding

This study was funded through BHLBI Intramural Grant Number NHLBI-CSB-(HL)-2013-013-JML. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

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Authors and Affiliations

  1. Division of Cardiology, Children’s National Health System, Washington DC, USA

    Bryan Siegel, Laura Olivieri & Christopher F. Spurney

  2. Center for Genetic Medicine Research, Children’s National Health System, Washington DC, USA

    Heather Gordish-Dressman & Christopher F. Spurney

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  1. Bryan Siegel
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  2. Laura Olivieri
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  3. Heather Gordish-Dressman
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  4. Christopher F. Spurney
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Correspondence to Bryan Siegel.

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Conflict of interest

Bryan Siegel declares that he has no conflict of interest. Laura Olivieri declares that she has no conflict of interest. Heather Gordish-Dressman declares that she has no conflict of interest. Christopher Spurney declares that he has no conflict of interest.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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Informed consent was obtained from all individual participants included in the study.

Research Involving Animal and Human Rights

This article does not contain any studies with animals performed by any of the authors.

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Siegel, B., Olivieri, L., Gordish-Dressman, H. et al. Myocardial Strain Using Cardiac MR Feature Tracking and Speckle Tracking Echocardiography in Duchenne Muscular Dystrophy Patients. Pediatr Cardiol 39, 478–483 (2018). https://doi.org/10.1007/s00246-017-1777-4

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  • DOI: https://doi.org/10.1007/s00246-017-1777-4

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