Yonsei Med J.  2016 Jul;57(4):900-904. 10.3349/ymj.2016.57.4.900.

Early Marker of Myocardial Deformation in Children with Duchenne Muscular Dystrophy Assessed Using Echocardiographic Myocardial Strain Analysis

Affiliations
  • 1Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea. lucyeun@yuhs.ac
  • 2Department of Rehabilitation Medicine, Yonsei University College of Medicine, Seoul, Korea.

Abstract

PURPOSE
As cardiomyopathy is more prevalent and currently the leading cause of death in Duchenne muscular dystrophy (DMD), early detection of myocardial involvement is important. The purpose of this study was to analyze myocardial strain in DMD children, for the possibility of early detection of myocardial dysfunction.
MATERIALS AND METHODS
We reviewed medical records of DMD patients who were >10 years of age (15.6±1.6 years, 12.5-18 years), from March 2013 to June 2014. Data of 24 DMD children who underwent echocardiography with three-layer specific myocardial strain were compared with 24 controls (age: 9.3±4.0 years, 5.5-17 years).
RESULTS
Epicardial longitudinal strain was lower in DMD (DMD: -9.3±3.8%; control: -12.3±4.3%; p=0.012). Radial strain (DMD: 24.1±11.1%; control: 37.3±25.9%; p=0.027) and strain rate (SR) (DMD: 1.68±0.91; control: 2.42±0.84; p=0.006) on parasternal short axis view were lower in DMD. Circumferential strains in the endocardium (DMD: -17.5±4.7%; control: -24.2±5.3%; p<0.001), myocardium (DMD: -12.7±3.8%; control: -18.0±4.0%; p<0.001), and epicardium (DMD: -8.4±4.0%; control: -12.2±5.0%; p=0.006) were significantly decreased in DMD. Circumferential SRs were lower in the endocardial (DMD: -1.46±0.38; control: -1.78±0.27; p=0.002) and myocardial layers (DMD: 1.02±0.27; control: -1.28±0.22; p=0.001).
CONCLUSION
In DMD patients, deteriorations in myocardial circumferential strain might be an indicator for predicting cardiomyopathy.

Keyword

Duchenne muscular dystrophy; children; myocardial strain; cardiomyopathies

MeSH Terms

Adolescent
Cardiomyopathies/*diagnostic imaging/*etiology
Case-Control Studies
Child
Child, Preschool
Early Diagnosis
*Echocardiography
Female
Humans
Male
Muscular Dystrophy, Duchenne/*complications/*diagnostic imaging
Predictive Value of Tests

Reference

1. Deconinck N, Dan B. Pathophysiology of duchenne muscular dystrophy: current hypotheses. Pediatr Neurol. 2007; 36:1–7.
Article
2. Finsterer J, Stöllberger C. The heart in human dystrophinopathies. Cardiology. 2003; 99:1–19.
Article
3. Spurney CF. Cardiomyopathy of Duchenne muscular dystrophy: current understanding and future directions. Muscle Nerve. 2011; 44:8–19.
Article
4. de Kermadec JM, Bécane HM, Chénard A, Tertrain F, Weiss Y. Prevalence of left ventricular systolic dysfunction in Duchenne muscular dystrophy: an echocardiographic study. Am Heart J. 1994; 127:618–623.
Article
5. Manzur AY, Kinali M, Muntoni F. Update on the management of Duchenne muscular dystrophy. Arch Dis Child. 2008; 93:986–990.
Article
6. Kim DH, Kang SW, Choi WA. Home mechanical ventilation in South Korea. Yonsei Med J. 2014; 55:1729–1735.
Article
7. Hor KN, Kissoon N, Mazur W, Gupta R, Ittenbach RF, Al-Khalidi HR, et al. Regional circumferential strain is a biomarker for disease severity in duchenne muscular dystrophy heart disease: a cross-sectional study. Pediatr Cardiol. 2015; 36:111–119.
Article
8. Adamu U, Schmitz F, Becker M, Kelm M, Hoffmann R. Advanced speckle tracking echocardiography allowing a three-myocardial layer-specific analysis of deformation parameters. Eur J Echocardiogr. 2009; 10:303–308.
Article
9. Leitman M, Lysiansky M, Lysyansky P, Friedman Z, Tyomkin V, Fuchs T, et al. Circumferential and longitudinal strain in 3 myocardial layers in normal subjects and in patients with regional left ventricular dysfunction. J Am Soc Echocardiogr. 2010; 23:64–70.
Article
10. Ryan TD, Taylor MD, Mazur W, Cripe LH, Pratt J, King EC, et al. Abnormal circumferential strain is present in young Duchenne muscular dystrophy patients. Pediatr Cardiol. 2013; 34:1159–1165.
Article
11. Helm PA, Younes L, Beg MF, Ennis DB, Leclercq C, Faris OP, et al. Evidence of structural remodeling in the dyssynchronous failing heart. Circ Res. 2006; 98:125–132.
Article
12. Bogaert J, Rademakers FE. Regional nonuniformity of normal adult human left ventricle. Am J Physiol Heart Circ Physiol. 2001; 280:H610–H620.
Article
13. Zhang Q, Fang F, Liang YJ, Xie JM, Wen YY, Yip GW, et al. A novel multi-layer approach of measuring myocardial strain and torsion by 2D speckle tracking imaging in normal subjects and patients with heart diseases. Int J Cardiol. 2011; 147:32–37.
Article
14. Finder JD, Birnkrant D, Carl J, Farber HJ, Gozal D, Iannaccone ST, et al. Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med. 2004; 170:456–465.
15. Duboc D, Meune C, Lerebours G, Devaux JY, Vaksmann G, Bécane HM. Effect of perindopril on the onset and progression of left ventricular dysfunction in Duchenne muscular dystrophy. J Am Coll Cardiol. 2005; 45:855–857.
Article
16. Duboc D, Meune C, Pierre B, Wahbi K, Eymard B, Toutain A, et al. Perindopril preventive treatment on mortality in Duchenne muscular dystrophy: 10 years' follow-up. Am Heart J. 2007; 154:596–602.
Article
17. Jefferies JL, Eidem BW, Belmont JW, Craigen WJ, Ware SM, Fernbach SD, et al. Genetic predictors and remodeling of dilated cardiomyopathy in muscular dystrophy. Circulation. 2005; 112:2799–2804.
Article
Full Text Links
  • YMJ
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr