Pediatric Cardiology

, Volume 39, Issue 8, pp 1614–1619 | Cite as

Evaluation of Early Left Ventricular Dysfunction in Patients with Duchenne Muscular Dystrophy Using Two-Dimensional Speckle Tracking Echocardiography and Tissue Doppler Imaging

  • Min-Jung ChoEmail author
  • Ji-Won Lee
  • JeSang Lee
  • Yong Beom Shin
Original Article


Although progressive cardiac dysfunction is the leading cause of death in patients with Duchenne muscular dystrophy (DMD), their cardiac function measured by conventional echocardiography has been generally interpreted as normal at a young age. We aimed to determine whether two-dimensional speckle tracking echocardiography (STE) or tissue Doppler imaging (TDI) could be used for early identification and detection of cardiac dysfunction in young patients with DMD. Thirteen pediatric patients (mean age, 9.69 ± 2.2 years) with DMD and 26 age-matched healthy children (mean age, 9.65 ± 2.2 years) were included in the study. All patients were examined via conventional echocardiography, TDI, and STE. Standard echocardiographic measurements of left ventricular (LV) systolic and diastolic function were obtained. Myocardial velocities including peak-systolic and early- and late-diastolic myocardial velocities were calculated in longitudinal direction in the interventricular septum, using TDI. Speckle tracking analyses were performed by acquiring apical four-, three-, and two-chamber views with the highest possible frame rates. Conventional parameters were similar between the two groups, but heart rates were higher in patients with DMD than in controls. The results of LV diastolic function evaluated using TDI showed that annular peak velocity during early diastole (e′; 10.9 ± 1.7 vs. 14.6 ± 1.7 cm/s), e′/a′ ratio (2.0 ± 0.5 vs. 3.0 ± 0.5), E/e′ ratio (9.4 ± 1.4 vs. 7.3 ± 0.8), and myocardial performance index (0.46 ± 0.05 vs. 0.36 ± 0.06) of the mitral septal annulus among patients with DMD differed significantly from those of healthy children. A significant decrease in global longitudinal systolic strain was found in patients with DMD (− 16.6 ± 3.7 vs. − 21.2 ± 2.1), with a marked decrease in the LV basal inferolateral and basal inferior walls. In young patients with DMD who have global normal systolic function, reductions in systolic deformation parameters as well as reduced early diastolic myocardial velocities can be detected particularly in the basal inferolateral LV walls. The prognostic significance of these findings warrants further longitudinal follow-up.


Muscular dystrophy Cardiomyopathy Pediatric Echocardiography Strain 



This study was funded by Biomedical Research Institute Grant, Pusan National University Hospital.

Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflict of interest to disclose.

Ethical Approval

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.

Informed Consent

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


  1. 1.
    Melacini P, Vianello A, Villanova C et al (1996) Cardiac and respiratory involvement in advanced stage Duchenne muscular dystrophy. Neuromuscul Disord 6:367–376CrossRefGoogle Scholar
  2. 2.
    Finsterer J, Stöllberger C (2003) The heart in human dystrophinopathies. Cardiology 99:1–19CrossRefGoogle Scholar
  3. 3.
    Muntoni F (2003) Cardiac complications of childhood myopathies. J Child Neurol 18:191–202CrossRefGoogle Scholar
  4. 4.
    Mertens L, Ganame J, Claus P et al (2008) Early regional myocardial dysfunction in young patients with Duchenne muscular dystrophy. J Am Soc Echocardiogr 21:1049–1054CrossRefGoogle Scholar
  5. 5.
    Lang RM, Bierig M, Devereux RB et al (2005) Recommendations for chamber quantification: a report from the American Society of Echocardiography’s Guidelines and Standards Committee and the Chamber Quantification Writing Group, developed in conjunction with the European Association of Echocardiography, a branch of the European Society of Cardiology. J Am Soc Echocardiogr 18:1440–1463CrossRefGoogle Scholar
  6. 6.
    Cerqueira MD, Weissman NJ, Dilsizian V et al (2002) Standardized myocardial segmentation and nomenclature for tomographic imaging of the heart: a statement for healthcare professionals from the Cardiac Imaging Committee of the Council on Clinical Cardiology of the American Heart Association. Circulation 105:539–542CrossRefGoogle Scholar
  7. 7.
    Eidem BW, McMahon CJ, Cohen RR et al (2004) Impact of cardiac growth on Doppler tissue imaging velocities: a study in healthy children. J Am Soc Echocardiogr 17:212–221CrossRefGoogle Scholar
  8. 8.
    Ciafaloni E, Moxley RT (2008) Treatment options for Duchenne muscular dystrophy. Curr Treat Options Neurol 10:86–93CrossRefGoogle Scholar
  9. 9.
    Shabanian R, Aboozari M, Kiani A et al (2011) Myocardial performance index and atrial ejection force in patients with Duchenne’s muscular dystrophy. Echocardiography 28:1088–1094CrossRefGoogle Scholar
  10. 10.
    Mori K, Hayabuchi Y, Inoue M et al (2007) Myocardial strain imaging for early detection of cardiac involvement in patients with Duchenne’s progressive muscular dystrophy. Echocardiography 24:598–608CrossRefGoogle Scholar
  11. 11.
    Ashford MW, Liu W, Lin SJ et al (2005) Occult cardiac contractile dysfunction in dystrophin-deficient children revealed by cardiac magnetic resonance strain imaging. Circulation 112:2462–2467CrossRefGoogle Scholar
  12. 12.
    Ogata H, Nakatani S, Ishikawa Y et al (2007) Myocardial strain changes in Duchenne muscular dystrophy without overt cardiomyopathy. Int J Cardiol 115:190–195CrossRefGoogle Scholar
  13. 13.
    Yamamoto T, Tanaka H, Matsumoto K et al (2013) Utility of transmural myocardial strain profile for prediction of early left ventricular dysfunction in patients with Duchenne muscular dystrophy. Am J Cardiol 111:902–907CrossRefGoogle Scholar
  14. 14.
    Nahum J, Bensaid A, Dussault C et al (2010) Impact of longitudinal myocardial deformation on the prognosis of chronic heart failure patients. Circ Cardiovasc Imaging 3:249–256CrossRefGoogle Scholar
  15. 15.
    Damy T, Margarit L, Noroc A et al (2012) Prognostic impact of sleep-disordered breathing and its treatment with nocturnal ventilation for chronic heart failure. Eur J Heart Fail 14:1009–1019CrossRefGoogle Scholar
  16. 16.
    Taqatqa A, Bokowski J, Al-Kubaisi M et al (2016) The use of speckle tracking echocardiography for early detection of myocardial dysfunction in patients with Duchenne muscular dystrophy. Pediatr Cardiol 37:1422–1428CrossRefGoogle Scholar
  17. 17.
    Bilchick KC, Salerno M, Plitt D et al (2011) Prevalence and distribution of regional scar in dysfunctional myocardial segments in Duchenne muscular dystrophy. J Cardiovasc Magn Reson 13:20CrossRefGoogle Scholar
  18. 18.
    Giatrakos N, Kinali M, Stephens D et al (2006) Cardiac tissue velocities and strain rate in the early detection of myocardial dysfunction of asymptomatic boys with Duchenne’s muscular dystrophy: relationship to clinical outcome. Heart 92:840–842CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Pediatrics, Biomedical Research Institute of Pusan National University HospitalPusan National University HospitalBusanRepublic of Korea
  2. 2.Department of Radiology, Biomedical Research Institute of Pusan National University HospitalPusan National University HospitalBusanRepublic of Korea
  3. 3.Department of Rehabilitation Medicine, Biomedical Research Institute of Pusan National University HospitalPusan National University HospitalBusanRepublic of Korea

Personalised recommendations