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

Abstract

BACKGROUND:

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.

PATIENTS:

39 male DMD patients aged 6-20 years were included. 6 patients were still ambulatory, 21 patients received corticosteroid therapy.

METHODS:

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).

RESULTS:

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).

DISCUSSION:

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.

CONCLUSION:

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: S Dittrich, M Tuerk, G Haaker, V Greim, A Buchholz, B Burkhardt, A Fujak, R Trollmann, A Schmid, R Schroeder
• Journal: Klinische Padiatrie (Klin Padiatr) Vol. 227 Issue 4 Pg. 225-31 (Jul 2015) ISSN: 1439-3824 [Electronic] Germany
• PMID: 26058601 (Publication Type: Journal Article)
• Copyright: © Georg Thieme Verlag KG Stuttgart · New York.

Chemical References

• Contrast Media
• Heterocyclic Compounds
• Organometallic Compounds
• Natriuretic Peptide, Brain
• gadolinium 1,4,7,10-tetraazacyclododecane-N,N',N'',N'''-tetraacetate

Topics

• Adolescent
• Autonomic Nervous System Diseases (diagnosis, physiopathology)
• Cardiomyopathies (diagnosis, physiopathology)
• Child
• Contrast Media (pharmacokinetics)
• Diagnostic Imaging
• Echocardiography, Doppler
• Elasticity Imaging Techniques
• Electrocardiography
• Electrocardiography, Ambulatory
• Heart Rate (physiology)
• Hemodynamics (physiology)
• Heterocyclic Compounds (pharmacokinetics)
• Humans
• Magnetic Resonance Imaging
• Male
• Muscular Dystrophy, Duchenne (diagnosis, physiopathology)
• Myocardial Contraction (physiology)
• Natriuretic Peptide, Brain (blood)
• Organometallic Compounds (pharmacokinetics)
• Reference Values
• Young Adult