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van Bockel EA, Lind JS, Zijlstra JG, et al. Cardiac assessment of patients with late stage Duchenne muscular dystrophy. Neth Heart J. 2009;17(6):232-7doi: 10.1007/BF03086253.
van Bockel, E. A., Lind, J. S., Zijlstra, J. G., Wijkstra, P. J., Meijer, P. M., van den Berg, M. P., Slart, R. H., Aarts, L. P., & Tulleken, J. E. (2009). Cardiac assessment of patients with late stage Duchenne muscular dystrophy. Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation, 17(6), 232-7. https://doi.org/10.1007/BF03086253
van Bockel, E A P, et al. "Cardiac assessment of patients with late stage Duchenne muscular dystrophy." Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation vol. 17,6 (2009): 232-7. doi: https://doi.org/10.1007/BF03086253
van Bockel EA, Lind JS, Zijlstra JG, Wijkstra PJ, Meijer PM, van den Berg MP, Slart RH, Aarts LP, Tulleken JE. Cardiac assessment of patients with late stage Duchenne muscular dystrophy. Neth Heart J. 2009 Jun;17(6):232-7. doi: 10.1007/BF03086253. PMID: 19789685; PMCID: PMC2711248.
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Neth Heart J. 2009 Jun;17(6):232-7. doi: 10.1007/BF03086253.

Cardiac assessment of patients with late stage Duchenne muscular dystrophy.

Netherlands heart journal : monthly journal of the Netherlands Society of Cardiology and the Netherlands Heart Foundation

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

Affiliations

  1. Department of Intensive Care, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands.

PMID: 19789685 PMCID: PMC2711248 DOI: 10.1007/BF03086253

Abstract

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.).

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