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Fulminant myocarditis

Abstract

Fulminant myocarditis is an inflammatory process that occurs in the myocardium and causes acute-onset heart failure. If patients with fulminant myocarditis are aggressively supported in a timely manner, nearly all can have an excellent recovery. In this Review, we discuss the clinical and histological distinguishing features of fulminant myocarditis and contrast this disease entity with nonfulminant myocarditis. The epidemiology, pathophysiology, clinical presentation, methods of diagnosis, management options and prognosis of fulminant myocarditis are reviewed in detail.

Key Points

  • Myocarditis could account for up to 10% of acute-onset heart failure cases; viral infections are responsible in the majority of instances

  • The pathophysiology of myocarditis involves both direct, virally mediated myocyte injury and immune-mediated tissue injury

  • Patients with fulminant myocarditis often present with cardiogenic shock and multiorgan failure; several clinical and laboratory findings enable the practicing physician to differentiate fulminant from nonfulminant myocarditis

  • Endomyocardial biopsy serves a critical role in the management of fulminant myocarditis and is an essential diagnostic tool to help differentiate myocarditis from giant cell myocarditis and necrotizing eosinophilic myocarditis

  • Patients with fulminant myocarditis should be managed with aggressive inotropic support with or without placement of an intra-aortic balloon pump; if the patient does not respond rapidly to aggressive supportive therapy, insertion of a ventricular assist device should be considered at an early stage

  • With aggressive supportive care, complete ventricular recovery occurs in the majority of patients with fulminant myocarditis; however, even after ventricular recovery, standard heart-failure medications should be administered indefinitely

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Figure 1: Pathogenesis of viral myocarditis can be divided into four phases.
Figure 2: Histologic image of an endomyocardial biopsy from a patient with giant cell myocarditis.
Figure 3: Histologic image of an endomyocardial biopsy from a patient with cardiac sarcoidosis.
Figure 4: Histologic classification of fulminant myocarditis by the Dallas criteria.59
Figure 5: A clinical algorithm that outlines the management of acute-onset severe heart failure.
Figure 6: Kaplan–Meier curve that shows unadjusted transplantation-free survival among patients with acute fulminant or nonfulminant myocarditis.

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Acknowledgements

Désirée Lie, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited continuing medical education activity associated with this article.The authors would like to acknowledge Dr Thomas E Rogers, Professor in Pathology at University of Texas Southwestern Medical Center, Dallas, TX, USA, for his invaluable help in obtaining the histologic images of endomyocardial biopsy slides.

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Correspondence to Pradeep PA Mammen.

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Gupta, S., Markham, D., Drazner, M. et al. Fulminant myocarditis. Nat Rev Cardiol 5, 693–706 (2008). https://doi.org/10.1038/ncpcardio1331

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