title: Myocardial inflammation in Duchenne Muscular Dystrophy as a precipitating factor for heart failure: a prospective study.
abstract: BACKGROUND
  In patients with Duchenne Muscular Dystrophy (DMD), the absent or diminished dystrophin leads to progressive skeletal muscle and heart failure. We evaluated the role of myocardial inflammation as a precipitating factor in the development of heart failure in DMD.
  
  
  METHODS
  20 DMD patients (aged 15-18 yrs) and 20 age-matched healthy volunteers were studied and followed-up for 2 years. Evaluation of myocarditis with cardiovascular magnetic resonance imaging (CMR) was performed using STIR T2-weighted (T2W), T1-weighted (T1W) before and after contrast media and late enhanced images (LGE). Left ventricular volumes and ejection fraction were also calculated. Myocardial biopsy was performed in patients with positive CMR and immunohistologic and polymerase chain reaction (PCR) analysis was employed.
  
  
  RESULTS
  In DMD patients, left ventricular end-diastolic volume (LVEDV) was not different compared to controls. Left ventricular end-systolic volume (LVESV) was higher (45.1 +/- 6.6 vs. 37.3 +/- 3.8 ml, p < 0.001) and left ventricular ejection fraction (LVEF) was lower (53.9 +/- 2.1 vs. 63 +/- 2.4%, p < 0.001). T2 heart/skeletal muscle ratio and early T1 ratio values in DMD patients presented no difference compared to controls. LGE areas were identified in six DMD patients. In four of them with CMR evidence of myocarditis, myocardial biopsy was performed. Active myocarditis was identified in one and healing myocarditis in three using immunohistology. All six patients with CMR evidence of myocarditis had a rapid deterioration of left ventricular function during the next year.
  
  
  CONCLUSIONS
  DMD patients with myocardial inflammation documented by CMR had a rigorous progression to heart failure.
authors.affiliation: A Cardiology Department, Onassis Cardiac Surgery Center, Athens, Greece. soma13@otenet.gr
authors.forename: Sophie
authors.initials: S
authors.lastname: Mavrogeni
authors.forename: Antigoni
authors.initials: A
authors.lastname: Papavasiliou
authors.forename: Kostas
authors.initials: K
authors.lastname: Spargias
authors.forename: Pantelis
authors.initials: P
authors.lastname: Constandoulakis
authors.forename: George
authors.initials: G
authors.lastname: Papadopoulos
authors.forename: Evangelos
authors.initials: E
authors.lastname: Karanasios
authors.forename: Dimitris
authors.initials: D
authors.lastname: Georgakopoulos
authors.forename: Genovefa
authors.initials: G
authors.lastname: Kolovou
authors.forename: Eftichia
authors.initials: E
authors.lastname: Demerouti
authors.forename: Spyridon
authors.initials: S
authors.lastname: Polymeros
authors.forename: Loukas
authors.initials: L
authors.lastname: Kaklamanis
authors.forename: Anastasios
authors.initials: A
authors.lastname: Magoutas
authors.forename: Evangelia
authors.initials: E
authors.lastname: Papadopoulou
authors.forename: Vyron
authors.initials: V
authors.lastname: Markussis
authors.forename: Dennis V
authors.initials: DV
authors.lastname: Cokkinos
mesh_terms: D000293:Adolescent; D001706:Biopsy / Q000379:methods; D016022:Case-Control Studies; D003287:Contrast Media; D004279:DNA, Viral / Q000235:genetics / Q000378:metabolism; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006333:Heart Failure / Q000209:etiology* / Q000473:pathology / Q000821:virology; D006346:Heart Septum / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000150:complications*; D009205:Myocarditis / Q000150:complications* / Q000209:etiology* / Q000473:pathology; D011446:Prospective Studies; D018709:Statistics, Nonparametric; D016277:Ventricular Function, Left
chemical_list: D003287:Contrast Media; D004279:DNA, Viral