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Abnormal Circumferential Strain is Present in Young Duchenne Muscular Dystrophy Patients

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Abstract

Advances in management of non-cardiac issues in Duchenne muscular dystrophy (DMD) have improved such that DMD-associated cardiac disease has become the leading cause of death for such patients. Cardiac dysfunction measured by standard transthoracic echocardiographic methods, e.g., fractional shortening (FS) and ejection fraction (EF), is rarely present during the first decade of life. The current study used transthoracic echocardiogram (TTE) to assess strain (ε), an indicator of regional ventricular function, in young DMD patients. A retrospective review of the TTE database was performed. TTE results from DMD patients <8 years (n = 63) performed during 2009 to 2010 were compared with TTE results from an unaffected control group (n = 61). Feature tracking analysis software was used to measure total circumferential strain (ε cc) as well as segmental ε cc based on the American Society of Echocardiography 16-segment model. Although there were no differences in FS, the absolute value for left-ventricular (LV) ε cc at the mid-chamber level was decreased in DMD (−21.7 % ± 3.8 % vs. −19.8 % ± 4.2 %, p < 0.01; unaffected vs. DMD). Segmental ε cc was similarly affected in the anteroseptal segment (−23.0 % ± 6.1 % vs. −18.9 % ± 7.0 %, p = 0.001; controls vs. DMD), the inferior segment (−20.7 % ± 5.16 % vs. −17.7 % ± 6.1 %, p = 0.003; controls vs. DMD), and the inferolateral segment (−18.3 % ± 6.2 % vs. −15.9 % ± 6.7 %, p = 0.04; controls vs. DMD). In the present study we demonstrate both total and segmental LV ε cc (anteroseptal, inferior, and inferolateral segments) abnormalities at the mid-chamber level in a large group of young DMD patients with normal FS. These novel findings substantiate that the disease process is present and results in abnormal myocardial function before standard measures detect global dysfunction.

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Abbreviations

ACEi:

Angiotensin converting enzyme inhibitor

CMR:

Cardiac magnetic resonance imaging

DMD:

Duchenne muscular dystrophy

ε :

Strain

ε cc :

Circumferential strain

EF:

Ejection fraction

FS:

Fractional shortening

ICC:

Intra-class correlation coefficients

LV:

Left ventricle, left ventricular

TTE:

Transthoracic echocardiogram

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Acknowledgments

The authors thank the sonographers in the Heart Institute at the Cincinnati Children’s Hospital Medical Center for their work performing the studies used in this investigation. Additional thanks are due to the Heart Institute Research Core for assistance in study design and approval.

Conflict of interest

The authors report no conflict of interest. No payment was given to any author to specifically produce this manuscript.

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Authors and Affiliations

  1. Division of Pediatric Cardiology, The Heart Institute, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Avenue, MLC 2003, Cincinnati, OH, 45229-3039, USA

    Thomas D. Ryan, Michael D. Taylor, Jesse Pratt, Eileen C. King, Kathleen Lao, Michelle A. Grenier & John L. Jefferies

  2. The Heart and Vascular Center at the Christ Hospital, 2139 Auburn Avenue, Cincinnati, OH, 45219, USA

    Wojciech Mazur & John L. Jefferies

  3. The Heart Center, Nationwide Children’s Hospital, 700 Children’s Drive, Columbus, OH, 43205, USA

    Linda H. Cripe & Kan N. Hor

  4. The Herma Heart Center, Children’s Hospital of Wisconsin, 9000 West Wisconsin Avenue, Milwaukee, WI, 53226, USA

    D. Woodrow Benson

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  1. Thomas D. Ryan
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Correspondence to Thomas D. Ryan.

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Ryan, T.D., Taylor, M.D., Mazur, W. et al. Abnormal Circumferential Strain is Present in Young Duchenne Muscular Dystrophy Patients. Pediatr Cardiol 34, 1159–1165 (2013). https://doi.org/10.1007/s00246-012-0622-z

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  • DOI: https://doi.org/10.1007/s00246-012-0622-z

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