Abstract
BackgroundIn 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.Methods20 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.ResultsIn 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.ConclusionsDMD patients with myocardial inflammation documented by CMR had a rigorous progression to heart failure.
Highlights
In patients with Duchenne Muscular Dystrophy (DMD), the absent or diminished dystrophin leads to progressive skeletal muscle and heart failure
Our aim was to evaluate a population of DMD patients, suspected for myocarditis, using cardiovascular magnetic resonance imaging (CMR) and myocardial biopsy, in order to examine the hypothesis that myocardial inflammation could be a precipitating factor for heart failure in these patients
In DMD patients, left ventricular end-diastolic volume (LVEDV) was not different compared to controls (96.9 ± 12.9 vs. 101 ± 13.2 ml, p:NS)
Summary
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. The absent or diminished dystrophin leads to progressive skeletal muscle and heart failure [1]. The use of a combination of STIR T2-weighted (T2W), T1-weighted (T1W) before and after contrast media and late enhanced images (LGE) can accurately diagnose the presence of myocardial inflammation missed by other imaging techniques [5]. Relative myocardial enhancement and LGE detect myocarditis with a sensitivity of 80% and a specificity of 68% and a sensitivity of 44% and a specificity of 100%, respectively
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