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Case Reports
. 2018 Oct 1;3(10):1006-1010.
doi: 10.1001/jamacardio.2018.2695.

Myocarditis in Duchenne Muscular Dystrophy After Changing Steroids

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Free PMC article
Case Reports

Myocarditis in Duchenne Muscular Dystrophy After Changing Steroids

Abdul Rahman A Abutaleb et al. JAMA Cardiol. .
Free PMC article

Abstract

Importance: Cardiac dysfunction is a leading cause of morbidity and mortality in Duchenne muscular dystrophy (DMD). This case highlights the importance of steroids in treating cardiac complications of DMD and the dangers of discontinuing or switching between steroid classes.

Objective: To recognize the presentation of acute myocardial inflammation, or dystrophinitis, in DMD, which presents as myocarditis and to treat the myocardial inflammation and dilated cardiomyopathy associated with DMD through guideline-directed medical therapy, steroids, and serial surveillance for cardiac dysfunction.

Design, setting, and participant: A case report of an 18-year-old patient with DMD and with steroid withdrawal-induced myocarditis followed up for 3 years to observe for cardiac function recovery and the natural history of cardiomyopathy in DMD, who was hospitalized in the cardiac care unit and followed up between November 3, 2016, and March 27, 2017.

Exposures: Switching from deflazacort to underdosed prednisone for 7 days.

Main outcomes and measures: Increased myocardial inflammation, edema, and fibrosis after stopping deflazacort abruptly.

Results: An 18-year-old male patient with DMD presented to the emergency department with acute-onset chest pain. Ischemic changes were present on electrocardiogram, and elevated cardiac enzymes were detected. Depressed cardiac function and potential evidence of inflammation were seen on cardiac magnetic resonance (CMR) imaging, characterized by elevated T2 values and late gadolinium enhancement. These findings were all consistent with acute myocarditis but without a viral prodrome. Several days prior to presentation, the patient's deflazacort was abruptly discontinued and converted to an equivalent dose of prednisone. After restarting deflazacort, his symptoms improved, and subsequent CMR showed resolution of myocardial edema and improved left ventricular function.

Conclusions and relevance: This case highlights adverse effects associated with changing between corticosteroid classes in DMD cardiomyopathy and also demonstrates the utility of CMR in detecting myocardial inflammation and monitoring response to treatment.

Conflict of interest statement

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.

Figures

Figure 1.
Figure 1.. ST Elevations in I-III, avF, V6 With Reciprocal Changes
Electrocardiogram of patient with Duchenne muscular dystrophy (DMD) who presented with substernal chest pain.
Figure 2.
Figure 2.. Normal Angiography of the Right Coronary Artery and Left Coronary Artery, Left Anterior Descending, and Left Circumflex Arteries
Coronary angiogram of patient with Duchenne muscular dystrophy who presented with substernal chest pain.
Figure 3.
Figure 3.. Cardiac Magnetic Resonance Imaging (MRI) on Presentation and 4-Month Follow-up Showing a Reduced Left Ventricular Ejection Fraction (LVEF) (42%) and Lateral Wall Hypokinesis
Myocardial edema in septal and lateral walls with elevated T2 values (57 milliseconds, normal reference, 40-55 millisecond) and the presence of late gadolinium enhancement (LGE) in the septum and basal to midlateral walls are consistent with acute myocarditis. Cardiac MRI 4 months later (B) showed improved myocardial function (LVEF 52%). Redemonstration of LGE in the basal to midlateral walls consistent with fibrosis and T2 values normalized to 42-49 milliseconds, indicating resolution of edema.

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