Pediatric Cardiology

, Volume 34, Issue 5, pp 1159–1165

Abnormal Circumferential Strain is Present in Young Duchenne Muscular Dystrophy Patients

  • Thomas D. Ryan
  • Michael D. Taylor
  • Wojciech Mazur
  • Linda H. Cripe
  • Jesse Pratt
  • Eileen C. King
  • Kathleen Lao
  • Michelle A. Grenier
  • John L. Jefferies
  • D. Woodrow Benson
  • Kan N. Hor
Original Article

DOI: 10.1007/s00246-012-0622-z

Cite this article as:
Ryan, T.D., Taylor, M.D., Mazur, W. et al. Pediatr Cardiol (2013) 34: 1159. doi:10.1007/s00246-012-0622-z

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 (εcc) as well as segmental εcc based on the American Society of Echocardiography 16-segment model. Although there were no differences in FS, the absolute value for left-ventricular (LV) εcc 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 εcc 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 εcc (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.

Keywords

Cardiomyopathy Duchenne muscular dystrophy Strain Echocardiography 

Abbreviations

ACEi

Angiotensin converting enzyme inhibitor

CMR

Cardiac magnetic resonance imaging

DMD

Duchenne muscular dystrophy

ε

Strain

εcc

Circumferential strain

EF

Ejection fraction

FS

Fractional shortening

ICC

Intra-class correlation coefficients

LV

Left ventricle, left ventricular

TTE

Transthoracic echocardiogram

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Thomas D. Ryan
    • 1
  • Michael D. Taylor
    • 1
  • Wojciech Mazur
    • 2
  • Linda H. Cripe
    • 3
  • Jesse Pratt
    • 1
  • Eileen C. King
    • 1
  • Kathleen Lao
    • 1
  • Michelle A. Grenier
    • 1
  • John L. Jefferies
    • 1
    • 2
  • D. Woodrow Benson
    • 4
  • Kan N. Hor
    • 3
  1. 1.Division of Pediatric Cardiology, The Heart InstituteCincinnati Children’s Hospital Medical CenterCincinnatiUSA
  2. 2.The Heart and Vascular Center at the Christ HospitalCincinnatiUSA
  3. 3.The Heart CenterNationwide Children’s HospitalColumbusUSA
  4. 4.The Herma Heart CenterChildren’s Hospital of WisconsinMilwaukeeUSA

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