Advertisement

Pediatric Radiology

, Volume 46, Issue 10, pp 1399–1408 | Cite as

Comparison of left ventricular function assessment between echocardiography and MRI in Duchenne muscular dystrophy

  • Sujatha Buddhe
  • Mark Lewin
  • Aaron Olson
  • Mark Ferguson
  • Brian D. Soriano
Original Article

Abstract

Background

Cardiomyopathy in Duchenne muscular dystrophy (DMD) is associated with death in approximately 40% of patients. Echocardiography is routinely used to assess left ventricular (LV) function; however, it has limitations in these patients.

Objective

We compared echocardiographic measures of cardiac function assessment to cardiac MRI.

Materials and methods

We included children and young adults with DMD who had MRI performed between January 2010 and July 2015. We measured echocardiographic and MRI parameters of function assessment, including strain. Presence of late gadolinium enhancement (LGE) was assessed by MRI. Subjects were divided into two groups based on MRI left ventricular ejection fraction (LVEF): group I, LVEF ≥55% and group II, LVEF <55%.

Results

We included 41 studies in 33 subjects, with 25 in group I and 16 in group II. Mean age of subjects was 13.6 ± 2.8 years and mean duration between echocardiogram and MRI was 7.6 ± 4.1 months. Only 8 of 16 (50%) patients in group II had diminished function on echocardiogram. Echocardiographic images were suboptimal in 16 subjects (39%). Overall, echocardiographic parameters had weak correlation with MRI-derived ejection fraction percentage. MRI-derived myocardial strain assessment has better correlation with MRI ejection fraction as compared to echocardiography-derived strain parameters.

Conclusion

Echocardiography-based ventricular functional assessment has weak correlation with MRI parameters in children and young adults with Duchenne muscular dystrophy. While this correlation improves in the subset of subjects with adequate echocardiographic image quality, it remains modest and potentially suboptimal for clinical management. Accordingly, we conclude that MRI should be performed routinely and early in children with DMD, not only for LGE imaging but also for functional assessment.

Keywords

Children Duchenne muscular dystrophy Echocardiography Magnetic resonance imaging Strain analysis 

Notes

Compliance with ethical standards

Conflicts of interest

None

References

  1. 1.
    Biggar WD (2006) Duchenne muscular dystrophy. Pediatr Rev 27:83–88CrossRefPubMedGoogle Scholar
  2. 2.
    Gilroy J, Cahalan JL, Berman R et al (1963) Cardiac and pulmonary complications in Duchenne’s progressive muscular dystrophy. Circulation 27:484–493CrossRefPubMedGoogle Scholar
  3. 3.
    Muntoni F (2003) Cardiac complications of childhood myopathies. J Child Neurol 18:191–202CrossRefPubMedGoogle Scholar
  4. 4.
    Pearson CM (1963) Muscular dystrophy. Review and recent observations. Am J Med 35:632–645CrossRefPubMedGoogle Scholar
  5. 5.
    Frankel KA, Rosser RJ (1976) The pathology of the heart in progressive muscular dystrophy: epimyocardial fibrosis. Hum Pathol 7:375–386CrossRefPubMedGoogle Scholar
  6. 6.
    English KM, Gibbs JL (2006) Cardiac monitoring and treatment for children and adolescents with neuromuscular disorders. Dev Med Child Neurol 48:231–235CrossRefPubMedGoogle Scholar
  7. 7.
    van Bockel EA, Lind JS, Zijlstra JG et al (2009) Cardiac assessment of patients with late stage Duchenne muscular dystrophy. Neth Hear J 17:232–237CrossRefGoogle Scholar
  8. 8.
    Spurney CF, McCaffrey FM, Cnaan A et al (2015) Feasibility and reproducibility of echocardiographic measures in children with muscular dystrophies. J Am Soc Echocardiogr 28:999–1008CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Mazur W, Hor KN, Germann JT et al (2012) Patterns of left ventricular remodeling in patients with Duchenne muscular dystrophy: a cardiac MRI study of ventricular geometry, global function, and strain. Int J Cardiovasc Imaging 28:99–107CrossRefPubMedGoogle Scholar
  10. 10.
    Verhaert D, Richards K, Rafael-Fortney JA et al (2011) Cardiac involvement in patients with muscular dystrophies: magnetic resonance imaging phenotype and genotypic considerations. Circ Cardiovasc Imaging 4:67–76CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Silva MC, Meira ZM, Gurgel Giannetti J et al (2007) Myocardial delayed enhancement by magnetic resonance imaging in patients with muscular dystrophy. J Am Coll Cardiol 49:1874–1879CrossRefPubMedGoogle Scholar
  12. 12.
    Hor KN, Taylor MD, Al-Khalidi HR et al (2013) Prevalence and distribution of late gadolinium enhancement in a large population of patients with Duchenne muscular dystrophy: effect of age and left ventricular systolic function. J Cardiovasc Magn Reson 15:107CrossRefPubMedPubMedCentralGoogle Scholar
  13. 13.
    Mertens L, Ganame J, Claus P et al (2008) Early regional myocardial dysfunction in young patients with Duchenne muscular dystrophy. J Am Soc Echocardiogr 21:1049–1054CrossRefPubMedGoogle Scholar
  14. 14.
    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 24:598–608CrossRefPubMedGoogle Scholar
  15. 15.
    Ryan TD, Taylor MD, Mazur W et al (2013) Abnormal circumferential strain is present in young Duchenne muscular dystrophy patients. Pediatr Cardiol 34:1159–1165CrossRefPubMedGoogle Scholar
  16. 16.
    Takano H, Fujii Y, Yugeta N et al (2011) Assessment of left ventricular regional function in affected and carrier dogs with Duchenne muscular dystrophy using speckle tracking echocardiography. BMC Cardiovasc Disord 11:23CrossRefPubMedPubMedCentralGoogle Scholar
  17. 17.
    Lai WW, Gauvreau K, Rivera ES et al (2008) Accuracy of guideline recommendations for two-dimensional quantification of the right ventricle by echocardiography. Int J Cardiovasc Imaging 24:691–698CrossRefPubMedGoogle Scholar
  18. 18.
    Walcher T, Steinbach P, Spiess J et al (2011) Detection of long-term progression of myocardial fibrosis in Duchenne muscular dystrophy in an affected family: a cardiovascular magnetic resonance study. Eur J Radiol 80:115–119CrossRefPubMedGoogle Scholar
  19. 19.
    Sasaki K, Sakata K, Kachi E et al (1998) Sequential changes in cardiac structure and function in patients with Duchenne type muscular dystrophy: a two-dimensional echocardiographic study. Am Heart J 135:937–944CrossRefPubMedGoogle Scholar
  20. 20.
    Brunklaus A, Parish E, Muntoni F et al (2015) The value of cardiac MRI versus echocardiography in the pre-operative assessment of patients with Duchenne muscular dystrophy. Eur J Paediatr Neurol 19:395–401CrossRefPubMedGoogle Scholar
  21. 21.
    Hor KN, Kissoon N, Mazur W et al (2015) Regional circumferential strain is a biomarker for disease severity in duchenne muscular dystrophy heart disease: a cross-sectional study. Pediatr Cardiol 36:111–119CrossRefPubMedGoogle Scholar
  22. 22.
    Grady RM, Teng H, Nichol MC et al (1997) Skeletal and cardiac myopathies in mice lacking utrophin and dystrophin: a model for Duchenne muscular dystrophy. Cell 90:729–738CrossRefPubMedGoogle Scholar
  23. 23.
    Ashford MW Jr, Liu W, Lin SJ et al (2005) Occult cardiac contractile dysfunction in dystrophin-deficient children revealed by cardiac magnetic resonance strain imaging. Circulation 112:2462–2467CrossRefPubMedGoogle Scholar
  24. 24.
    Li P, Meng H, Liu SZ et al (2012) Quantification of left ventricular mechanics using vector-velocity imaging, a novel feature tracking algorithm, applied to echocardiography and cardiac magnetic resonance imaging. Chin Med J 125:2719–2727PubMedGoogle Scholar
  25. 25.
    Williams LK, Urbano-Moral JA, Rowin EJ et al (2013) Velocity vector imaging in the measurement of left ventricular myocardial mechanics on cardiac magnetic resonance imaging: correlations with echocardiographically derived strain values. J Am Soc Echocardiogr 26:1153–1162CrossRefPubMedGoogle Scholar
  26. 26.
    Hor KN, Gottliebson WM, Carson C et al (2010) Comparison of magnetic resonance feature tracking for strain calculation with harmonic phase imaging analysis. JACC Cardiovasc Imaging 3:144–151CrossRefPubMedGoogle Scholar
  27. 27.
    Puchalski MD, Williams RV, Askovich B et al (2009) Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy? Int J Cardiovasc Imaging 25:57–63CrossRefPubMedGoogle Scholar
  28. 28.
    Buss SJ, Breuninger K, Lehrke S et al (2015) Assessment of myocardial deformation with cardiac magnetic resonance strain imaging improves risk stratification in patients with dilated cardiomyopathy. Eur Heart J Cardiovasc Imaging 16:307–315CrossRefPubMedGoogle Scholar
  29. 29.
    Ntusi NA, Piechnik SK, Francis JM et al (2015) Diffuse myocardial fibrosis and inflammation in rheumatoid arthritis: insights from CMR T1 mapping. JACC Cardiovasc Imaging 8:526–536CrossRefPubMedGoogle Scholar
  30. 30.
    Soslow JH, Markham LW, Saville B et al (2014) Left ventricular function by echocardiography correlates poorly with cardiac MRI measures in Duchenne muscular dystrophy. J Cardiovasc Magn Reson 16:P306CrossRefPubMedCentralGoogle Scholar
  31. 31.
    Moody WE, Taylor RJ, Edwards NC et al (2015) Comparison of magnetic resonance feature tracking for systolic and diastolic strain and strain rate calculation with spatial modulation of magnetization imaging analysis. J Magn Reson Imaging 41:1000–1012CrossRefPubMedGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2016

Authors and Affiliations

  • Sujatha Buddhe
    • 1
  • Mark Lewin
    • 1
  • Aaron Olson
    • 1
  • Mark Ferguson
    • 2
  • Brian D. Soriano
    • 1
  1. 1.Division of Cardiology, Department of PediatricsUniversity of Washington School of Medicine and Seattle Children’s HospitalSeattleUSA
  2. 2.Department of RadiologyUniversity of Washington School of Medicine and Seattle Children’s HospitalSeattleUSA

Personalised recommendations