title: 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.
  
  
  METHODS
  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).
  
  
  CONCLUSIONS
  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.
  
  
  CONCLUSIONS
  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.affiliation: Pediatric Cardiology, University Hospital, Erlangen, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Dittrich
authors.affiliation: Neurology, University Hospital, Erlangen, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Tuerk
authors.affiliation: Neuropathology, University Hospital, Erlangen, Germany.
authors.forename: G
authors.initials: G
authors.lastname: Haaker
authors.affiliation: Pediatric Cardiology, University Hospital, Erlangen, Germany.
authors.forename: V
authors.initials: V
authors.lastname: Greim
authors.affiliation: Clinical Trials Unit, University of Freiburg, Freiburg, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Buchholz
authors.affiliation: Pediatric Cardiology, University Medical Center, Freiburg, Germany.
authors.forename: B
authors.initials: B
authors.lastname: Burkhardt
authors.affiliation: Orthopedics, University Hospital, Erlangen, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Fujak
authors.affiliation: Pediatrics, University Hospital, Erlangen, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Trollmann
authors.affiliation: Radiology, University Hospital, Erlangen, Germany.
authors.forename: A
authors.initials: A
authors.lastname: Schmid
authors.affiliation: Neurology, University Hospital, Erlangen, Germany.
authors.forename: R
authors.initials: R
authors.lastname: Schroeder
mesh_terms: D000293:Adolescent; D001342:Autonomic Nervous System Diseases / Q000175:diagnosis / Q000503:physiopathology; D009202:Cardiomyopathies / Q000175:diagnosis* / Q000503:physiopathology*; D002648:Child; D003287:Contrast Media / Q000493:pharmacokinetics; D003952:Diagnostic Imaging; D015150:Echocardiography, Doppler; D054459:Elasticity Imaging Techniques; D004562:Electrocardiography; D015716:Electrocardiography, Ambulatory; D006339:Heart Rate / Q000502:physiology*; D006439:Hemodynamics / Q000502:physiology; D006571:Heterocyclic Compounds / Q000493:pharmacokinetics; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000175:diagnosis* / Q000503:physiopathology*; D009200:Myocardial Contraction / Q000502:physiology*; D020097:Natriuretic Peptide, Brain / Q000097:blood*; D009942:Organometallic Compounds / Q000493:pharmacokinetics; D012016:Reference Values; D055815:Young Adult
chemical_list: D003287:Contrast Media; D006571:Heterocyclic Compounds; D009942:Organometallic Compounds; D020097:Natriuretic Peptide, Brain; C050823:gadolinium 1,4,7,10-tetraazacyclododecane-N,N',N'',N'''-tetraacetate