Top Things to Know: Recognition and Initial Management of Fulminant Myocarditis

Published: January 06, 2020

  1. Fulminant myocarditis (FM), an acute and severe inflammation of the myocardium, is often underdiagnosed and is associated with an exceptionally high mortality.
  2. Cardinal features include rapidly progressive heart failure and cardiogenic shock as well as electrical instability leading to sudden death.
  3. Early evaluation and management are important to distinguish FM from other forms of acute circulatory compromise including ischemic heart disease, stress-induced cardiomyopathy, acute cardiac tamponade, and cardiomyopathy in pregnancy.
  4. This statement is meant to educate frontline health care providers to consider and identify FM at its earliest stages using the most relevant advanced diagnostic modalities to provide appropriate treatment and optimize outcomes.
  5. The etiology of FM may include lymphocytic myocarditis, giant cell myocarditis and acute necrotizing eosinophilic myocarditis, as well those resulting from novel cancer therapies such as immune checkpoint inhibitors.
  6. Multi-modality cardiovascular imaging including echocardiography, gadolinium contrast-enhanced cardiac magnetic resonance (CMR) and contrast-enhanced cardiac computed tomography (CT) play a key role in establishing a diagnosis and determining tissue-level pathologies.
  7. Endomyocardial biopsy can be helpful in the setting of unexplained, new-onset heart failure associated with hemodynamic compromise, a dilated left ventricle, new atrial or ventricular arrhythmias, or a failure to respond to standard care within a few weeks of diagnosis.
  8. Patients with FM often present in cardiogenic shock accompanied by atrial and ventricular tachyarrhythmias, bradyarrhythmia due to heart block, syncope and sudden cardiac death.
  9. FM may respond to specific immunomodulatory therapy, including high dose steroids and other therapeutic agents, depending on the etiology subtype.
  10. Early recognition of FM, institution of circulatory support, and maintenance of end-organ function can result in favorable outcomes.

Citation


Kociol RD, Cooper LT, Fang JC, Moslehi JJ, Pang PS, Sabe MA, Shah RV, Sims DB, Thiene G, Vardeny O, on behalf of the American Heart Association Heart Failure and Transplantation Committee of the Council on Clinical Cardiology. Recognition and initial management of fulminant myocarditis: a scientific statement from the American Heart Association [published online ahead of print January 6, 2020]. Circulation. doi: 10.1161/CIR.0000000000000745.