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. 2009 Jun;17(6):232-7.
doi: 10.1007/BF03086253.

Cardiac assessment of patients with late stage Duchenne muscular dystrophy

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
Affiliations

Cardiac assessment of patients with late stage Duchenne muscular dystrophy

E A P van Bockel et al. Neth Heart J. 2009 Jun.

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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Figures

Figure 1
Figure 1
Left ventricular ejection fraction (%) measured by multigated cardiac radionuclide ventriculography (MUGA) grouped by preserved and depressed function and NT-proBNP levels in individual patients.
Figure 2
Figure 2
Left ventricular ejection fraction (%) measured by multigated cardiac radionuclide ventriculography (MUGA) versus left ventricular function (LVF) measured by echocardiography and NT-proBNP
See this image and copyright information in PMC

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