Background: Idiopathic inflammatory myopathies (IIMs) are autoimmune diseases that are characterized by muscle injury. These disorders can cause cardiomyopathy and heart failure, myocarditis, and arrhythmias. However, only a few cases of cardiac arrest as a result of IIMs have been previously reported.
Case summary: A 46-year-old male presented with an out-of-hospital ventricular fibrillation cardiac arrest. A diagnosis of IIM had been made through a muscle biopsy performed 2 years before presentation. The patient had a positive anti-nuclear antibody but negative myositis-specific antibodies. His initial symptoms of IIM were mild and consisted of myalgias. His only cardiac symptoms were minor palpitations that occurred 3 years prior to the cardiac arrest, with a negative Holter monitor test result at that time. His cardiac catheterization was normal. He was suspected to have myocarditis, and a rheumatologist was consulted, following which the patient was initiated on intravenous immunoglobulin (IVIG). Cardiac magnetic resonance imaging demonstrated evidence of chronic myocarditis and an ejection fraction of 44%. He was initiated on goal-directed medical therapy for heart failure. A VVI implantable cardioverter defibrillator was implanted for secondary prevention. He was discharged and prescribed additional immunosuppression including further IVIG infusions, prednisone taper and rituximab infusions.
Discussion: Our case demonstrates that cardiac arrest in IIM is not only plausible, but can be the first major cardiac manifestation of the disease. When a diagnosis of IIM is made, patients require a thorough assessment of cardiac symptomatology and a low threshold for additional cardiac investigations.
Keywords: Autoimmune inflammatory myopathies; Cardiac risk; Cardiomyopathy; Case report; Myocarditis; Ventricular fibrillation.
© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.