Early treatment with corticosteroids quickly reversed ST-segment elevation and troponin levels in a 9-year-old male with Duchenne muscular dystrophy (DMD)-associated myocardial injury, according to a recently published case report in the Journal of Cardiothoracic Surgery.
DMD cardiomyopathy, characterized by muscle inflammation and the deposition of fibrotic tissue, is currently the leading cause of death among patients with DMD, the researchers noted. The early detection and treatment of cardiac muscle damage is vital for the overall outcome of patients with the condition, they added.
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Although, in most cases, cardiac disease is progressive rather than acute, many experts agree that acute cases are followed by muscle regeneration that could delay the development of subsequent myocardial dysfunction, the study team noted. Some studies suggest that corticosteroid therapy could improve cardiac function in patients with DMD; however, more evidence is required on the subject, they continued.
“Acute chest pain attacks with elevated troponin in patients with DMD without coronary artery disease may indicate acute myocardial injury, a sign of progression to cardiomyopathy,” the authors wrote.
The case involved a 9-year-old male with DMD complaining of acute-onset oppressive thoracic pain. The patient was currently under treatment with 18 mg deflazacort OD, and an echocardiogram performed approximately a year before admission was unremarkable.
A physical admission exam revealed elevated blood pressure, tachypnea, and gallop rhythm with a murmur. A 12-lead electrocardiogram revealed ST elevation in the inferior leads with reciprocal depression in V1 to V3. Laboratory workup was remarkable for elevated troponin, CK-MB, and brain natriuretic peptide.
An echocardiogram revealed several areas of hypokinesia and a 46% ejection fraction. The acute coronary syndrome was ruled out through computed tomography (CT) angiography. Finally, magnetic resonance imaging showed findings consistent with acute myocarditis leading to the diagnosis of acute myocardial injury associated with DMD.
The patient was started on anticongestive therapy, and deflazacort was replaced with methylprednisolone at 2mg/kg/ day. Physicians noted the absence of chest pain and improvement of the electrocardiographic, laboratory, and echocardiographic parameters within the next 5 days.
“Prospective studies with larger series are needed to prove the efficacy of steroids in the treatment of these acute myocardial injury episodes with chest pain, troponin elevation, and ECG changes in DMD patients,” the authors concluded.
Oğuz M, Gürses D, Ufuk F, et al. Successful treatment of acute myocardial injury of Duchenne muscular dystrophy with steroids: a case report. J Cardiothorac Surg. Published online March 3, 2023. doi:10.1186/s13019-023-02159-8