Feasibility and Reproducibility of Echocardiographic Measures in Children with Muscular Dystrophies

Christopher F. Spurney, Francis M. McCaffrey, Avital Cnaan, Lauren P. Morgenroth, Sunil J. Ghelani, Heather Gordish-Dressman, Adrienne Arrieta, Anne M. Connolly, Timothy E. Lotze, Craig M McDonald, Robert T. Leshner, Paula R. Clemens

Research output: Contribution to journalArticle

22 Citations (Scopus)

Abstract

Background Cardiac disease is a major cause of death in patients with muscular dystrophies. The use of feasible and reproducible echocardiographic measures of cardiac function is critical to advance the field of therapeutics for dystrophic cardiomyopathy. Methods Participants aged 8 to 18 years with genetically confirmed Duchenne muscular dystrophy (DMD), Becker muscular dystrophy, or limb-girdle muscular dystrophy were enrolled at five centers, and standardized echocardiographic examinations were performed. Measures of systolic and diastolic function and speckle-tracking echocardiography-derived cardiac strain were reviewed independently by two central readers. Furthermore, echocardiographic measures from participants with DMD were compared with those from retrospective age-matched control subjects from a single site to assess measures of myocardial function. Results Forty-eight participants (mean age, 13.3 ± 2.7 years) were enrolled. Shortening fraction had a greater interobserver correlation (intraclass correlation coefficient [ICC] = 0.63) compared with ejection fraction (ICC = 0.49). One reader could measure ejection fraction in only 53% of participants. Myocardial performance index measured by pulse-wave Doppler and Doppler tissue imaging showed similar ICCs (0.55 and 0.54). Speckle-tracking echocardiography showed a high ICC (0.96). Focusing on participants with DMD (n = 33), significantly increased mitral A-wave velocities, lower E/A ratios, and lower Doppler tissue imaging mitral lateral E′ velocities were observed compared with age-matched control subjects. Speckle-tracking echocardiography demonstrated subclinical myocardial dysfunction with decreased average circumferential and longitudinal strain in three distinct subgroups: participants with DMD with normal shortening fractions, participants with DMD aged < 13 years, and participants with DMD with myocardial performance index scores < 0.40 compared with control subjects. Conclusions In a muscular dystrophy cohort, assessment of cardiac function is feasible and reproducible using shortening fraction, diastolic measures, and myocardial performance index. Cardiac strain measures identified early myocardial disease in patients with DMD.

Original languageEnglish (US)
Pages (from-to)999-1008
Number of pages10
JournalJournal of the American Society of Echocardiography
Volume28
Issue number8
DOIs
StatePublished - Aug 1 2015

Fingerprint

Duchenne Muscular Dystrophy
Muscular Dystrophies
Echocardiography
Cardiomyopathies
Limb-Girdle Muscular Dystrophies
Pulse
Cause of Death
Heart Diseases

Keywords

  • Cardiac strain
  • Cardiomyopathy
  • Echocardiography
  • Muscular dystrophy

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

Cite this

Spurney, C. F., McCaffrey, F. M., Cnaan, A., Morgenroth, L. P., Ghelani, S. J., Gordish-Dressman, H., ... Clemens, P. R. (2015). Feasibility and Reproducibility of Echocardiographic Measures in Children with Muscular Dystrophies. Journal of the American Society of Echocardiography, 28(8), 999-1008. https://doi.org/10.1016/j.echo.2015.03.003

Feasibility and Reproducibility of Echocardiographic Measures in Children with Muscular Dystrophies. / Spurney, Christopher F.; McCaffrey, Francis M.; Cnaan, Avital; Morgenroth, Lauren P.; Ghelani, Sunil J.; Gordish-Dressman, Heather; Arrieta, Adrienne; Connolly, Anne M.; Lotze, Timothy E.; McDonald, Craig M; Leshner, Robert T.; Clemens, Paula R.

In: Journal of the American Society of Echocardiography, Vol. 28, No. 8, 01.08.2015, p. 999-1008.

Research output: Contribution to journalArticle

Spurney, CF, McCaffrey, FM, Cnaan, A, Morgenroth, LP, Ghelani, SJ, Gordish-Dressman, H, Arrieta, A, Connolly, AM, Lotze, TE, McDonald, CM, Leshner, RT & Clemens, PR 2015, 'Feasibility and Reproducibility of Echocardiographic Measures in Children with Muscular Dystrophies', Journal of the American Society of Echocardiography, vol. 28, no. 8, pp. 999-1008. https://doi.org/10.1016/j.echo.2015.03.003
Spurney, Christopher F. ; McCaffrey, Francis M. ; Cnaan, Avital ; Morgenroth, Lauren P. ; Ghelani, Sunil J. ; Gordish-Dressman, Heather ; Arrieta, Adrienne ; Connolly, Anne M. ; Lotze, Timothy E. ; McDonald, Craig M ; Leshner, Robert T. ; Clemens, Paula R. / Feasibility and Reproducibility of Echocardiographic Measures in Children with Muscular Dystrophies. In: Journal of the American Society of Echocardiography. 2015 ; Vol. 28, No. 8. pp. 999-1008.
@article{063421f6d5a24f2c924cc2fa6ae24209,
title = "Feasibility and Reproducibility of Echocardiographic Measures in Children with Muscular Dystrophies",
abstract = "Background Cardiac disease is a major cause of death in patients with muscular dystrophies. The use of feasible and reproducible echocardiographic measures of cardiac function is critical to advance the field of therapeutics for dystrophic cardiomyopathy. Methods Participants aged 8 to 18 years with genetically confirmed Duchenne muscular dystrophy (DMD), Becker muscular dystrophy, or limb-girdle muscular dystrophy were enrolled at five centers, and standardized echocardiographic examinations were performed. Measures of systolic and diastolic function and speckle-tracking echocardiography-derived cardiac strain were reviewed independently by two central readers. Furthermore, echocardiographic measures from participants with DMD were compared with those from retrospective age-matched control subjects from a single site to assess measures of myocardial function. Results Forty-eight participants (mean age, 13.3 ± 2.7 years) were enrolled. Shortening fraction had a greater interobserver correlation (intraclass correlation coefficient [ICC] = 0.63) compared with ejection fraction (ICC = 0.49). One reader could measure ejection fraction in only 53{\%} of participants. Myocardial performance index measured by pulse-wave Doppler and Doppler tissue imaging showed similar ICCs (0.55 and 0.54). Speckle-tracking echocardiography showed a high ICC (0.96). Focusing on participants with DMD (n = 33), significantly increased mitral A-wave velocities, lower E/A ratios, and lower Doppler tissue imaging mitral lateral E′ velocities were observed compared with age-matched control subjects. Speckle-tracking echocardiography demonstrated subclinical myocardial dysfunction with decreased average circumferential and longitudinal strain in three distinct subgroups: participants with DMD with normal shortening fractions, participants with DMD aged < 13 years, and participants with DMD with myocardial performance index scores < 0.40 compared with control subjects. Conclusions In a muscular dystrophy cohort, assessment of cardiac function is feasible and reproducible using shortening fraction, diastolic measures, and myocardial performance index. Cardiac strain measures identified early myocardial disease in patients with DMD.",
keywords = "Cardiac strain, Cardiomyopathy, Echocardiography, Muscular dystrophy",
author = "Spurney, {Christopher F.} and McCaffrey, {Francis M.} and Avital Cnaan and Morgenroth, {Lauren P.} and Ghelani, {Sunil J.} and Heather Gordish-Dressman and Adrienne Arrieta and Connolly, {Anne M.} and Lotze, {Timothy E.} and McDonald, {Craig M} and Leshner, {Robert T.} and Clemens, {Paula R.}",
year = "2015",
month = "8",
day = "1",
doi = "10.1016/j.echo.2015.03.003",
language = "English (US)",
volume = "28",
pages = "999--1008",
journal = "Journal of the American Society of Echocardiography",
issn = "0894-7317",
publisher = "Mosby Inc.",
number = "8",

}

TY - JOUR

T1 - Feasibility and Reproducibility of Echocardiographic Measures in Children with Muscular Dystrophies

AU - Spurney, Christopher F.

AU - McCaffrey, Francis M.

AU - Cnaan, Avital

AU - Morgenroth, Lauren P.

AU - Ghelani, Sunil J.

AU - Gordish-Dressman, Heather

AU - Arrieta, Adrienne

AU - Connolly, Anne M.

AU - Lotze, Timothy E.

AU - McDonald, Craig M

AU - Leshner, Robert T.

AU - Clemens, Paula R.

PY - 2015/8/1

Y1 - 2015/8/1

N2 - Background Cardiac disease is a major cause of death in patients with muscular dystrophies. The use of feasible and reproducible echocardiographic measures of cardiac function is critical to advance the field of therapeutics for dystrophic cardiomyopathy. Methods Participants aged 8 to 18 years with genetically confirmed Duchenne muscular dystrophy (DMD), Becker muscular dystrophy, or limb-girdle muscular dystrophy were enrolled at five centers, and standardized echocardiographic examinations were performed. Measures of systolic and diastolic function and speckle-tracking echocardiography-derived cardiac strain were reviewed independently by two central readers. Furthermore, echocardiographic measures from participants with DMD were compared with those from retrospective age-matched control subjects from a single site to assess measures of myocardial function. Results Forty-eight participants (mean age, 13.3 ± 2.7 years) were enrolled. Shortening fraction had a greater interobserver correlation (intraclass correlation coefficient [ICC] = 0.63) compared with ejection fraction (ICC = 0.49). One reader could measure ejection fraction in only 53% of participants. Myocardial performance index measured by pulse-wave Doppler and Doppler tissue imaging showed similar ICCs (0.55 and 0.54). Speckle-tracking echocardiography showed a high ICC (0.96). Focusing on participants with DMD (n = 33), significantly increased mitral A-wave velocities, lower E/A ratios, and lower Doppler tissue imaging mitral lateral E′ velocities were observed compared with age-matched control subjects. Speckle-tracking echocardiography demonstrated subclinical myocardial dysfunction with decreased average circumferential and longitudinal strain in three distinct subgroups: participants with DMD with normal shortening fractions, participants with DMD aged < 13 years, and participants with DMD with myocardial performance index scores < 0.40 compared with control subjects. Conclusions In a muscular dystrophy cohort, assessment of cardiac function is feasible and reproducible using shortening fraction, diastolic measures, and myocardial performance index. Cardiac strain measures identified early myocardial disease in patients with DMD.

AB - Background Cardiac disease is a major cause of death in patients with muscular dystrophies. The use of feasible and reproducible echocardiographic measures of cardiac function is critical to advance the field of therapeutics for dystrophic cardiomyopathy. Methods Participants aged 8 to 18 years with genetically confirmed Duchenne muscular dystrophy (DMD), Becker muscular dystrophy, or limb-girdle muscular dystrophy were enrolled at five centers, and standardized echocardiographic examinations were performed. Measures of systolic and diastolic function and speckle-tracking echocardiography-derived cardiac strain were reviewed independently by two central readers. Furthermore, echocardiographic measures from participants with DMD were compared with those from retrospective age-matched control subjects from a single site to assess measures of myocardial function. Results Forty-eight participants (mean age, 13.3 ± 2.7 years) were enrolled. Shortening fraction had a greater interobserver correlation (intraclass correlation coefficient [ICC] = 0.63) compared with ejection fraction (ICC = 0.49). One reader could measure ejection fraction in only 53% of participants. Myocardial performance index measured by pulse-wave Doppler and Doppler tissue imaging showed similar ICCs (0.55 and 0.54). Speckle-tracking echocardiography showed a high ICC (0.96). Focusing on participants with DMD (n = 33), significantly increased mitral A-wave velocities, lower E/A ratios, and lower Doppler tissue imaging mitral lateral E′ velocities were observed compared with age-matched control subjects. Speckle-tracking echocardiography demonstrated subclinical myocardial dysfunction with decreased average circumferential and longitudinal strain in three distinct subgroups: participants with DMD with normal shortening fractions, participants with DMD aged < 13 years, and participants with DMD with myocardial performance index scores < 0.40 compared with control subjects. Conclusions In a muscular dystrophy cohort, assessment of cardiac function is feasible and reproducible using shortening fraction, diastolic measures, and myocardial performance index. Cardiac strain measures identified early myocardial disease in patients with DMD.

KW - Cardiac strain

KW - Cardiomyopathy

KW - Echocardiography

KW - Muscular dystrophy

UR - http://www.scopus.com/inward/record.url?scp=84938553819&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84938553819&partnerID=8YFLogxK

U2 - 10.1016/j.echo.2015.03.003

DO - 10.1016/j.echo.2015.03.003

M3 - Article

C2 - 25906753

AN - SCOPUS:84938553819

VL - 28

SP - 999

EP - 1008

JO - Journal of the American Society of Echocardiography

JF - Journal of the American Society of Echocardiography

SN - 0894-7317

IS - 8

ER -