Cardiomyopathy in Duchenne Muscular Dystrophy: Current Value of Clinical, Electrophysiological and Imaging Findings in Children and Teenagers

Dittrich S, Türk M, Haaker G, Greim V, Buchholz A, Burkhardt B, Fujak A, Trollmann R, Schmid A, Schröder R (2015)

Publication Type: Journal article

Publication year: 2015

Journal

Klinische Pädiatrie Georg Thieme Verlag

Publisher: Georg Thieme Verlag

Book Volume: 227

Pages Range: 225-31

Journal Issue: 4

DOI: 10.1055/s-0034-1398689

Abstract

Progressive cardiomyopathy (CMP) is one main cause of death in DMD. This cross-sectional assessment of different cardiac diagnostic procedures focusses on preterm diagnosis of cardiac dysfunction.39 male DMD patients aged 6-20 years were included. 6 patients were still ambulatory, 21 patients received corticosteroid therapy.All patients were investigated by ECG, Holter ECG and heart rate variability (HRV), B-type natriuretic peptide (BNP), echocardiography (TTE), tissue Doppler Imaging (TD) and magnetic resonance imaging (MRI) with Late Gadolinium enhancement (LE) and segmental wall motion analysis (WMA).56% of the patients showed repolarization abnormalities and 76% altered HRV. Subnormal ventricular function was found in 25% by TTE and in 34% by MRI. TD differed from normal controls only in the apical septum. In MRI 89% of the patients showed different distribution and intensity of LE and WM restriction. The extent of LE was less in patients after steroid treatment (p<0.05).MRI with segmental LE- and WM-analysis seems to be superior to TTE and TD in exploring regional distribution and severity of damage of the myocardium. ECG and HRV abnormalities are common in DMD-patients but not tightly predictive for segmental and global left ventricular dysfunction. Targeted treatment of CMP in DMD needs prospective evaluation.A timely cardiac MRI is the most sensitive investigation for the identification of early myocardial changes in DMD which is a prerequisite for early interventions and therapeutic strategies.

Authors with CRIS profile

Sven Dittrich Professur für Kinderkardiologie Matthias Türk Lehrstuhl für Neuropathologie Regina Trollmann Professur für Kinder- u. Jugendmedizin Schwerpunkt Neuropädiatrie Andrea Schmid Lehrstuhl für Diagnostische Radiologie Rolf Schröder Professur für Neuropathologie

Involved external institutions

Albert-Ludwigs-Universität Freiburg DE Germany (DE)

How to cite

APA:

Dittrich, S., Türk, M., Haaker, G., Greim, V., Buchholz, A., Burkhardt, B.,... Schröder, R. (2015). Cardiomyopathy in Duchenne Muscular Dystrophy: Current Value of Clinical, Electrophysiological and Imaging Findings in Children and Teenagers. Klinische Pädiatrie, 227(4), 225-31. https://doi.org/10.1055/s-0034-1398689

MLA:

Dittrich, Sven, et al. "Cardiomyopathy in Duchenne Muscular Dystrophy: Current Value of Clinical, Electrophysiological and Imaging Findings in Children and Teenagers." Klinische Pädiatrie 227.4 (2015): 225-31.

BibTeX: Download