Pediatric Cardiology

, Volume 38, Issue 5, pp 1042–1048 | Cite as

Relationship Between Fragmented QRS Complexes and Cardiac Status in Duchenne Muscular Dystrophy: Multimodal Validation Using Echocardiography, Magnetic Resonance Imaging, and Holter Monitoring

  • Min-Jung Cho
  • Ji-Won Lee
  • JeSang Lee
  • Yong Bum Shin
  • Hyoung Doo Lee
Original Article

Abstract

The presence of fragmented QRS is a known marker of heterogeneous ventricular activation around the myocardial scar area. We validated whether fragmented QRS shows any association with ventricular dysfunction, fibrosis, or ventricular arrhythmias in patients with Duchenne muscular dystrophy (DMD). Thirty-seven patients with DMD were evaluated using electrocardiography (ECG), echocardiography, cardiac magnetic resonance, and 24-h Holter monitoring. Associations between fragmented QRS and ventricular dysfunction, fibrosis, or ventricular arrhythmia were investigated. Fragmented QRS complexes were present in 31 of 37 (83.7%) patients, and they were associated with a significantly lower left ventricular ejection fraction along with an increased left ventricular Tei index as evaluated by echocardiography, and more frequent ventricular arrhythmia as indicated using 24-hour Holter monitoring compared with patients without fragmented QRS. The number of leads with fragmented QRS correlated negatively with left ventricular ejection fraction both using echocardiography (r = −0.616) and CMR (r = −0.516). Further, the number of leads with fragmented QRS showed a significant correlation with several other echocardiographic measurements (mitral Em and Sm, and left ventricular Tei index), and 2-dimensional speckle-tracking echocardiography derived global left ventricular longitudinal strain. The frequency of ventricular arrhythmia observed using Holter monitoring showed a significant positive correlation with the frequency of fragmented QRS on ECG (r = 0.674). There was a positive trend of correlation between fragmented QRS and the amount of myocardial fibrosis as assessed by late gadolinium enhancement using CMR, but the statistical significance of the relationship was low (r = 0.433, p = 0.056). Fragmentation of QRS complexes is associated with degrees of left ventricular dysfunction, fibrosis, and ventricular arrhythmias in patients with DMD.

Keywords

Muscular dystrophy Cardiomyopathy QRS fragmentation Electrocardiography Ambulatory Echocardiography Cardiac magnetic resonance 

Notes

Funding

This study was funded by Biomedical Research Institute Grant (2015-06), 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.

References

  1. 1.
    Melacini P, Vianello A, Villanova C, Fanin M, Miorin M, Angelini C, Dalla Volta S (1996) Cardiac and respiratory involvement in advanced stage Duchenne muscular dystrophy. Neuromuscul Disord 6:367–376CrossRefPubMedGoogle Scholar
  2. 2.
    Finsterer J, Stöllberger C (2003) The heart in human dystrophinopathies. Cardiology 99:1–19CrossRefPubMedGoogle Scholar
  3. 3.
    Das MK, Khan B, Jacob S, Kumar A, Mahenthiran J (2006) Significance of a fragmented QRS complex versus a Q wave in patients with coronary artery disease. Circulation 113:2495–2501CrossRefPubMedGoogle Scholar
  4. 4.
    Homsi M, Alsayed L, Safadi B, Mahenthiran J, Das MK (2009) Fragmented QRS complexes on 12-lead ECG: a marker of cardiac sarcoidosis as detected by gadolinium cardiac magnetic resonance imaging. Ann Noninvasive Electrocardiol 14:319–326CrossRefPubMedGoogle Scholar
  5. 5.
    Lang RM, Bierig M, Devereux RB, Flachskampf FA, Foster E, Pellikka PA, Picard MH, Roman MJ, Seward J, Shanewise JS, Solomon SD, Spencer KT, Sutton MS, Stewart WJ, Group CQW, Committee ASoEsGaS, Echocardiography EAo (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 18:1440–1463CrossRefPubMedGoogle Scholar
  6. 6.
    de Bakker JM, van Capelle FJ, Janse MJ, Tasseron S, Vermeulen JT, de Jonge N, Lahpor JR (1993) Slow conduction in the infarcted human heart. ‘Zigzag’ course of activation. Circulation 88:915–926CrossRefPubMedGoogle Scholar
  7. 7.
    Frankel KA, Rosser RJ (1976) The pathology of the heart in progressive muscular dystrophy: epimyocardial fibrosis. Hum Pathol 7:375–386CrossRefPubMedGoogle Scholar
  8. 8.
    Canga A, Kocaman SA, Durakoğlugil ME, Cetin M, Erdoğan T, Kırış T, Erden M (2013) Relationship between fragmented QRS complexes and left ventricular systolic and diastolic functions. Herz 38:665–670CrossRefPubMedGoogle Scholar
  9. 9.
    Urheim S, Edvardsen T, Torp H, Angelsen B, Smiseth OA (2000) Myocardial strain by Doppler echocardiography. Validation of a new method to quantify regional myocardial function. Circulation 102:1158–1164CrossRefPubMedGoogle Scholar
  10. 10.
    Koyama J, Ray-Sequin PA, Falk RH (2003) Longitudinal myocardial function assessed by tissue velocity, strain, and strain rate tissue Doppler echocardiography in patients with AL (primary) cardiac amyloidosis. Circulation 107:2446–2452CrossRefPubMedGoogle Scholar
  11. 11.
    Dragulescu A, Mertens L, Friedberg MK (2013) Interpretation of left ventricular diastolic dysfunction in children with cardiomyopathy by echocardiography: problems and limitations. Circ Cardiovasc Imaging 6:254–261CrossRefPubMedGoogle Scholar
  12. 12.
    Shabanian R, Aboozari M, Kiani A, Seifirad S, Zamani G, Nahalimoghaddam A, Kocharian A (2011) Myocardial performance index and atrial ejection force in patients with Duchenne’s muscular dystrophy. Echocardiography 28:1088–1094CrossRefPubMedGoogle Scholar
  13. 13.
    Nazarian S, Bluemke DA, Lardo AC, Zviman MM, Watkins SP, Dickfeld TL, Meininger GR, Roguin A, Calkins H, Tomaselli GF, Weiss RG, Berger RD, Lima JA, Halperin HR (2005) Magnetic resonance assessment of the substrate for inducible ventricular tachycardia in nonischemic cardiomyopathy. Circulation 112:2821–2825CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Menon SC, Etheridge SP, Liesemer KN, Williams RV, Bardsley T, Heywood MC, Puchalski MD (2014) Predictive value of myocardial delayed enhancement in Duchenne muscular dystrophy. Pediatr Cardiol 35:1279–1285CrossRefPubMedGoogle Scholar
  15. 15.
    Gardner PI, Ursell PC, Fenoglio JJ, Wit AL (1985) Electrophysiologic and anatomic basis for fractionated electrograms recorded from healed myocardial infarcts. Circulation 72:596–611CrossRefPubMedGoogle Scholar
  16. 16.
    Park SJ, On YK, Kim JS, Park SW, Yang JH, Jun TG, Kang IS, Lee HJ, Choe YH, Huh J (2012) Relation of fragmented QRS complex to right ventricular fibrosis detected by late gadolinium enhancement cardiac magnetic resonance in adults with repaired tetralogy of fallot. Am J Cardiol 109:110–115CrossRefPubMedGoogle Scholar
  17. 17.
    Morita H, Kusano KF, Miura D, Nagase S, Nakamura K, Morita ST, Ohe T, Zipes DP, Wu J (2008) Fragmented QRS as a marker of conduction abnormality and a predictor of prognosis of Brugada syndrome. Circulation 118:1697–1704CrossRefPubMedGoogle Scholar
  18. 18.
    Peters S, Trümmel M, Koehler B (2008) QRS fragmentation in standard ECG as a diagnostic marker of arrhythmogenic right ventricular dysplasia-cardiomyopathy. Heart Rhythm 5:1417–1421CrossRefPubMedGoogle Scholar
  19. 19.
    Nomura A, Konno T, Fujita T, Tanaka Y, Nagata Y, Tsuda T, Hodatsu A, Sakata K, Nakamura H, Kawashiri MA, Fujino N, Yamagishi M, Hayashi K (2015) Fragmented QRS predicts heart failure progression in patients with hypertrophic cardiomyopathy. Circ J 79:136–143CrossRefPubMedGoogle Scholar
  20. 20.
    Mewton N, Liu CY, Croisille P, Bluemke D, Lima JA (2011) Assessment of myocardial fibrosis with cardiovascular magnetic resonance. J Am Coll Cardiol 57:891–903CrossRefPubMedGoogle Scholar
  21. 21.
    Ozawa K, Funabashi N, Takaoka H, Ueda M, Kobayashi Y (2014) Risk stratification using a combination of left ventricular fibrosis and number of morphological types of ventricular premature beats in cardiomyopathy subjects without obstructed coronary arteries. Int J Cardiol 176:236–239CrossRefPubMedGoogle Scholar
  22. 22.
    Kang KW, Janardhan AH, Jung KT, Lee HS, Lee MH, Hwang HJ (2014) Fragmented QRS as a candidate marker for high-risk assessment in hypertrophic cardiomyopathy. Heart Rhythm 11:1433–1440CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2017

Authors and Affiliations

  • Min-Jung Cho
    • 1
  • Ji-Won Lee
    • 2
  • JeSang Lee
    • 3
  • Yong Bum Shin
    • 3
  • Hyoung Doo Lee
    • 4
  1. 1.Department of Pediatrics, Biomedical Research Institute of Pusan National University HospitalPusan National University HospitalBusanRepublic of Korea
  2. 2.Department of RadiologyPusan National University HospitalBusanRepublic of Korea
  3. 3.Department of Rehabilitation MedicinePusan National University HospitalBusanRepublic of Korea
  4. 4.Pusan National University Children’s HospitalBusanRepublic of Korea

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