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. 2010 May 21:10:33.
doi: 10.1186/1471-2377-10-33.

Myocardial inflammation in Duchenne Muscular Dystrophy as a precipitating factor for heart failure: a prospective study

Sophie Mavrogeni  1 , Antigoni Papavasiliou, Kostas Spargias, Pantelis Constandoulakis, George Papadopoulos, Evangelos Karanasios, Dimitris Georgakopoulos, Genovefa Kolovou, Eftichia Demerouti, Spyridon Polymeros, Loukas Kaklamanis, Anastasios Magoutas, Evangelia Papadopoulou, Vyron Markussis, Dennis V Cokkinos
Affiliations

Myocardial inflammation in Duchenne Muscular Dystrophy as a precipitating factor for heart failure: a prospective study

Sophie Mavrogeni et al. BMC Neurol. .

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.

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Figures

Figure 1
Figure 1
Intramural late gadolinium enhanced area (LGE) in the intraventricular septum (arrows), indicative of active myocardial inflammation.
Figure 2
Figure 2
Epicardial late gadolinium enhanced area (LGE) in the inferior LV wall (arrows), indicative of healing myocarditis.
Figure 3
Figure 3
Pathology image (arrows), indicative of active myocardial inflammation (hematoxylin-eosin, ×200), corresponding to figure 1.
Figure 4
Figure 4
Pathology image (arrows), indicative of healing myocarditis (hematoxylin-eosin, ×200), corresponding to figure 2.
Figure 5
Figure 5
A Kaplan-Meier graph of the probability of heart failure between LGE+ (continuous line) and LGE- (dotted line) DMD patients.
See this image and copyright information in PMC

References

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