Myocardial inflammation in Duchenne Muscular Dystrophy as a precipitating factor for heart failure: a prospective study.
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.
Mavrogeni
Sophie
S
A Cardiology Department, Onassis Cardiac Surgery Center, Athens, Greece. soma13@otenet.gr
Papavasiliou
Antigoni
A
Spargias
Kostas
K
Constandoulakis
Pantelis
P
Papadopoulos
George
G
Karanasios
Evangelos
E
Georgakopoulos
Dimitris
D
Kolovou
Genovefa
G
Demerouti
Eftichia
E
Polymeros
Spyridon
S
Kaklamanis
Loukas
L
Magoutas
Anastasios
A
Papadopoulou
Evangelia
E
Markussis
Vyron
V
Cokkinos
Dennis V
DV
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
D003287:Contrast Media; D004279:DNA, Viral