Netherlands Heart Journal

, Volume 17, Issue 6, pp 232–237 | Cite as

Cardiac assessment of patients with late stage Duchenne muscular dystrophy

  • E. A. P. van Bockel
  • J. S.  Lind
  • J. G.  Zijlstra
  • P. J.  Wijkstra
  • P. M.  Meijer
  • M. P. van den Berg
  • R. H. J. A.  Slart
  • L. P. H. J.  Aarts
  • J. E.  Tulleken
Original article


Background. Duchenne muscular dystrophy (DMD) patients used to die mainly from pulmonary problems. However, as advances in respiratory care increase life expectancy, mortality due to cardiomyopathy rises. Echocardiography remains the standard diagnostic modality for cardiomyopathy in DMD patients, but is hampered by scoliosis and poor echocardiographic acoustic windows in adult DMD patients. Multigated cardiac radionuclide ventriculography (MUGA) does not suffer from these limitations. N-terminal proBNP (NTproBNP) has shown to be a diagnostic factor for heart failure. We present our initial experience with plasma NT-proBNP measurement in the routine screening and diagnosis of cardiomyopathy in adult mechanically ventilated DMD patients.

Methods. Retrospective study, 13 patients. Echocardiography classified left ventricular (LV) function as preserved or depressed. NT-proBNP was determined using immunoassay. LV ejection fraction (LVEF) was determined using MUGA.

Results. Median (range) NT-proBNP was 73 (25 to 463) ng/l. Six patients had an NT-proBNP >125 ng/l. Seven patients showed an LVEF <45% on MUGA. DMD patients with depressed LV function (n=4) as assessed by echocardiography had significantly higher median NT-proBNP than those (n=9) with preserved LV function: 346 (266 to 463) ng/l versus 69 (25 to 257) ng/l (p=0.003). NT-proBNP significantly correlated with depressed LV function on echocardiogram and with LVEF determined by MUGA.

Conclusion. Although image quality of MUGA is superior to echocardiography, the combination of echocardiography and NT-proBNP achieves similar results in the evaluation of left ventricular function and is less time consuming and burdensome for our patients. We advise to add NT-proBNP to echocardiography in the routine cardiac assessment of DMD patients. (Neth Heart J 2009;17:232–7.)

natriuretic peptides echocardiography multigated radionuclide ventriculography cardiomyopathy home mechanical ventilation Duchenne muscular dystrophy 


  1. 1.
    Bushby K, Muntoni F, Bourke JP. 107th ENMC international workshop: the management of cardiac involvement in muscular dystrophy and myotonic dystrophy. 7th–9th June 2002 Naarden, the Netherlands. Neuromuscul Disord 2003;13:166–72.Google Scholar
  2. 2.
    Finsterer J, Stollberger C. The heart in human dystrophinopathies. Cardiology 2003;99:1–19.Google Scholar
  3. 3.
    Silva MC, Meira ZM, Gurgel GJ, da Silva MM, Campos AF, Barbosa MM, et al. Myocardial delayed enhancement by magnetic resonance imaging in patients with muscular dystrophy. J Am Coll Cardiol 2007;49:1874–9.Google Scholar
  4. 4.
    Puchalski MD, Williams RV, Askovich B, Sower CT, Hor KH, Su JT, et al. Late gadolinium enhancement: precursor to cardiomyopathy in Duchenne muscular dystrophy? Int J Cardiovasc Imaging 2009;25:57–63.Google Scholar
  5. 5.
    Toussaint M, Steens M, Wasteels G, Soudon P. Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients. Eur Respir J 2006;28:549–55.Google Scholar
  6. 6.
    Yasuma F, Konagaya M, Sakai M, Kuru S, Kawamura T. A new lease on life for patients with Duchenne muscular dystrophy in Japan. Am J Med 2004;117:363.Google Scholar
  7. 7.
    Oguz D, Olgunturk R, Gucuyener K, Acikgoz GV, Tunaoglu FS. A comparison between MUGA and echocardiography in patients with muscular dystrophy in the early detection of cardiac involvement. Pediatr Cardiol 1998;19:150–4.Google Scholar
  8. 8.
    Silver MA, Maisel A, Yancy CW, McCullough PA, Burnett JC Jr, Francis GS, et al. BNP Consensus Panel 2004: A clinical approach for the diagnostic, prognostic, screening, treatment monitoring, and therapeutic roles of natriuretic peptides in cardiovascular diseases. Congest Heart Fail 2004;10(5 Suppl 3):1–30.Google Scholar
  9. 9.
    Maisel AS, Krishnaswamy P, Nowak RM, McCord J, Hollander JE, Duc P, et al. Rapid measurement of B-type natriuretic peptide in the emergency diagnosis of heart failure. N Engl J Med 2002; 347:161–7.Google Scholar
  10. 10.
    Mori K, Manabe T, Nii M, Hayabuchi Y, Kuroda Y, Tatara K. Plasma levels of natriuretic peptide and echocardiographic parameters in patients with Duchenne’s progressive muscular dystrophy. Pediatr Cardiol 2002;23:160–6.Google Scholar
  11. 11.
    Cheitlin MD, Armstrong WF, Aurigemma GP, Beller GA, Bierman FZ, Davis JL, et al. ACC/AHA/ASE 2003 guideline update for the clinical application of echocardiography-summary article: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (ACC/ AHA/ASE Committee to Update the 1997 Guidelines for the Clinical Application of Echocardiography). J Am Coll Cardiol 2003;42:954–70.Google Scholar
  12. 12.
    van der Vleuten PA, Slart RH, Tio RA, van der Horst IC, van Veldhuisen DJ, Dierckx RA et al. The feasibility of repeated left ventricular ejection fraction analysis with sequential single-dose radionuclide ventriculography. Nucl Med Commun 2005;26:711–5.Google Scholar
  13. 13.
    Demachi J, Kagaya Y, Watanabe J, Sakuma M, Ikeda J, Kakuta Y et al. Characteristics of the increase in plasma brain natriuretic peptide level in left ventricular systolic dysfunction, associated with muscular dystrophy in comparison with idiopathic dilated cardiomyopathy. Neuromuscul Disord 2004;14:732–9.Google Scholar
  14. 14.
    Ishikawa Y, Bach JR, Minami R. Cardioprotection for Duchenne’s muscular dystrophy. Am Heart J 1999;137:895–902.Google Scholar
  15. 15.
    Krauser DG, Lloyd-Jones DM, Chae CU, Cameron R, Anwaruddin S, Baggish AL, et al. Effect of body mass index on natriuretic peptide levels in patients with acute congestive heart failure: a ProBNP Investigation of Dyspnea in the Emergency Department (PRIDE) substudy. Am Heart J 2005;149:744–50.Google Scholar
  16. 16.
    Corrado G, Lissoni A, Beretta S, Terenghi L, Tadeo G, Foglia-Manzillo G, et al. Prognostic value of electrocardiograms, ventricular late potentials, ventricular arrhythmias, and left ventricular systolic dysfunction in patients with Duchenne muscular dystrophy. Am J Cardiol 2002;89:838–41.Google Scholar
  17. 17.
    Mertens L, Ganame J, Claus P, Goemans N, Thijs D, Eyskens B, et al. Early regional myocardial dysfunction in young patients with Duchenne muscular dystrophy. J Am Soc Echocardiogr 2008;21: 1049–54.Google Scholar
  18. 18.
    Mohyuddin T, Jacobs IB, Bahler RC. B-type natriuretic peptide and cardiac dysfunction in Duchenne muscular dystrophy. Int J Cardiol 2007;119:389–91.Google Scholar
  19. 19.
    Hoogerwaard EM, van der Wouw PA, Wilde AA, Bakker E, Ippel PF, Oosterwijk JC, et al. Cardiac involvement in carriers of Duchenne and Becker muscular dystrophy. Neuromuscul Disord 1999;9:347–51.Google Scholar
  20. 20.
    Adachi K, Kawai H, Saito M, Naruo T, Kimura C, Mine H, et al. Plasma levels of brain natriuretic peptide as an index for evaluation of cardiac function in female gene carriers of Duchenne muscular dystrophy. Intern Med 1997;36:497–500.Google Scholar
  21. 21.
    Duboc D, Meune C, Lerebours G, Devaux JY, Vaksmann G, Becane HM. Effect of perindopril on the onset and progression of left ventricular dysfunction in Duchenne muscular dystrophy. J Am Coll Cardiol 2005;45:855–7.Google Scholar
  22. 22.
    Ramaciotti C, Scott WA, Lemler MS, Haverland C, Iannaccone ST. Assessment of cardiac function in adolescents with Duchenne muscular dystrophy: importance of neurohormones. J Child Neurol 2002;17:191–4.Google Scholar

Copyright information

© Bohn Stafleu van Loghum 2009

Authors and Affiliations

  • E. A. P. van Bockel
    • 1
  • J. S.  Lind
  • J. G.  Zijlstra
  • P. J.  Wijkstra
  • P. M.  Meijer
  • M. P. van den Berg
  • R. H. J. A.  Slart
  • L. P. H. J.  Aarts
  • J. E.  Tulleken
  1. 1.

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