When Lightning Strikes
Fulminant Myocarditis in the Realm of Inflammatory Cardiomyopathies
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Before the advent of endomyocardial biopsy in the 1960s, myocarditis was known as an uncommon cause of acute and fatal heart failure. Biopsy and later cardiac magnetic resonance imaging (MRI) revealed that myocarditis was both more common and less lethal than originally described. With greater use of imaging and biopsy, the clinical spectrum of myocarditis broadened to include milder presentations such as pericarditis with elevated troponin values, nonsustained ventricular arrhythmias, and occasionally heart block.1 Patients who present with severe heart failure of <2-week symptom duration requiring parenteral inotropic or mechanical circulatory support are defined as having fulminant myocarditis. These relatively uncommon patients have a high risk in the near term of heart transplantation and death.2 The cause of fulminant myocarditis historically was often an enteroviral (eg, Coxsackie virus) infection. As enterovirus prevalence has waned, new causes of fulminant myocarditis, including H1N1 influenza,3 and drug-related eosinophilic myocarditis4,5 have emerged.
Fulminant myocarditis, as described by Lieberman et al6 in 1991, was a clinical and a pathological entity. Clinically, all subjects had a distinct viral prodrome and severe hemodynamic compromise. Pathologically, all cases had an active inflammatory infiltrate of lymphocytes and macrophages with myocyte necrosis. No subjects with fulminant myocarditis in the original description had giant cell myocarditis. Lymphocytic myocarditis that presents with a distinct viral …