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Presence of mechanical dyssynchrony in Duchenne muscular dystrophy.

Publication ,  Journal Article
Hor, KN; Wansapura, JP; Al-Khalidi, HR; Gottliebson, WM; Taylor, MD; Czosek, RJ; Nagueh, SF; Akula, N; Chung, ES; Benson, WD; Mazur, W
Published in: J Cardiovasc Magn Reson
February 2, 2011

BACKGROUND: Cardiac dysfunction in boys with Duchenne muscular dystrophy (DMD) is a leading cause of death. Cardiac resynchronization therapy (CRT) has been shown to dramatically decrease mortality in eligible adult population with congestive heart failure. We hypothesized that mechanical dyssynchrony is present in DMD patients and that cardiovascular magnetic resonance (CMR) may predict CRT efficacy. METHODS: DMD patients (n = 236) were stratified into 4 groups based on age, diagnosis of DMD, left ventricular (LV) ejection fraction (EF), and presence of myocardial fibrosis defined as positive late gadolinum enhancement (LGE) compared to normal controls (n = 77). Dyssynchrony indices were calculated based on timing of CMR derived circumferential strain (ecc). The calculated indices included cross-correlation delay (XCD), uniformity of strain (US), regional vector of variance (RVV), time to maximum strain (TTMS) and standard deviation (SD) of TTMS. Abnormal XCD value was defined as > normal + 2SD. US, RVV, TTMS and SD were calculated for patients with abnormal XCD. RESULTS: There was overall low prevalence of circumferential dyssynchrony in the entire DMD population; it increased to 17.1% for patients with abnormal EF and to 31.2% in the most advanced stage (abnormal EF with fibrosis). All but one DMD patient with mechanical dyssynchrony exhibited normal QRS duration suggesting absence of electrical dyssynchrony. The calculated US and RVV values (0.91 ± 0.09, 1.34 ± 0.48) indicate disperse rather than clustered dyssynchrony. CONCLUSION: Mechanical dyssynchrony is frequent in boys with end stage DMD-associated cardiac dysfunction. It is associated with normal QRS complex as well as extensive lateral fibrosis. Based on these findings, it is unlikely that this patient population will benefit from CRT.

Duke Scholars

Published In

J Cardiovasc Magn Reson

DOI

EISSN

1532-429X

Publication Date

February 2, 2011

Volume

13

Issue

1

Start / End Page

12

Location

England

Related Subject Headings

  • Young Adult
  • Ventricular Function, Left
  • Ventricular Dysfunction, Left
  • Stroke Volume
  • Severity of Illness Index
  • Retrospective Studies
  • Predictive Value of Tests
  • Patient Selection
  • Ohio
  • Nuclear Medicine & Medical Imaging
 

Citation

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Hor, K. N., Wansapura, J. P., Al-Khalidi, H. R., Gottliebson, W. M., Taylor, M. D., Czosek, R. J., … Mazur, W. (2011). Presence of mechanical dyssynchrony in Duchenne muscular dystrophy. J Cardiovasc Magn Reson, 13(1), 12. https://doi.org/10.1186/1532-429X-13-12
Hor, Kan N., Janaka P. Wansapura, Hussein R. Al-Khalidi, William M. Gottliebson, Michael D. Taylor, Richard J. Czosek, Sherif F. Nagueh, et al. “Presence of mechanical dyssynchrony in Duchenne muscular dystrophy.J Cardiovasc Magn Reson 13, no. 1 (February 2, 2011): 12. https://doi.org/10.1186/1532-429X-13-12.
Hor KN, Wansapura JP, Al-Khalidi HR, Gottliebson WM, Taylor MD, Czosek RJ, et al. Presence of mechanical dyssynchrony in Duchenne muscular dystrophy. J Cardiovasc Magn Reson. 2011 Feb 2;13(1):12.
Hor, Kan N., et al. “Presence of mechanical dyssynchrony in Duchenne muscular dystrophy.J Cardiovasc Magn Reson, vol. 13, no. 1, Feb. 2011, p. 12. Pubmed, doi:10.1186/1532-429X-13-12.
Hor KN, Wansapura JP, Al-Khalidi HR, Gottliebson WM, Taylor MD, Czosek RJ, Nagueh SF, Akula N, Chung ES, Benson WD, Mazur W. Presence of mechanical dyssynchrony in Duchenne muscular dystrophy. J Cardiovasc Magn Reson. 2011 Feb 2;13(1):12.
Journal cover image

Published In

J Cardiovasc Magn Reson

DOI

EISSN

1532-429X

Publication Date

February 2, 2011

Volume

13

Issue

1

Start / End Page

12

Location

England

Related Subject Headings

  • Young Adult
  • Ventricular Function, Left
  • Ventricular Dysfunction, Left
  • Stroke Volume
  • Severity of Illness Index
  • Retrospective Studies
  • Predictive Value of Tests
  • Patient Selection
  • Ohio
  • Nuclear Medicine & Medical Imaging