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Myocardial disease
Clinical presentation and diagnosis of myocarditis
  1. Alida L P Caforio1,
  2. Renzo Marcolongo2,
  3. Cristina Basso3,
  4. Sabino Iliceto1
  1. 1Cardiology, Department of Cardiological Thoracic and Vascular Sciences, University of Padua, Padua, Italy
  2. 2Clinical Immunology, Department of Medicine (DIMED), University of Padua, Padua, Italy
  3. 3Cardiovascular Pathology, Department of Cardiological, Thoracic and Vascular Sciences, University of Padua, Padua, Italy
  1. Correspondence to Dr Alida L P Caforio, Division of Cardiology, Department of Cardiological, Thoracic and Vascular Sciences, Centro ‘V. Gallucci’, University of Padova-Policlinico, Via Giustiniani, 2, Padova 35128, Italy; alida.caforio{at}unipd.it

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Learning objectives

  1. How to formulate the clinical suspicion of myocarditis.

  2. How to reach the diagnosis of ‘clinically suspected’ and ‘definite (biopsy-proven)’ myocarditis according to the European Society of Cardiology (ESC) 2013 Task Force criteria.

  3. How to interpret non-invasive and invasive diagnostic findings in the context of the heterogeneous clinical presentations of suspected myocarditis.

  4. How to assess prognosis.

Myocarditis represents a challenging diagnosis, mainly because there is no pathognomonic clinical presentation, and the disease may masquerade as a variety of non-inflammatory myocardial diseases. Thus, in the 1996 WHO/International Society and Federation of Cardiology (WHO/ISFC)1 and in the 2007 European Society of Cardiology (ESC) classifications of cardiomyopathies,2 as well as in the 2013 ESC myocarditis Task Force report,3 the disease is defined histologically as an inflammatory disease of the myocardium diagnosed on endomyocardial biopsy (EMB), based upon histological, immunological, immunohistochemical and molecular findings to detect possible infectious causes.1–6 The term inflammatory cardiomyopathy may be used for histologically confirmed myocarditis in association with cardiac dysfunction (box 1).1 Although EMB remains the diagnostic gold standard for myocarditis and it is the only tool that, at present, provides distinction of its aetiological forms,1–6 it is not widely used. This results in lack of certainty in the epidemiological impact and the natural history of the disease. Myocarditis may resolve spontaneously, recur or become chronic, leading about 1/3 of biopsy-proven cases to dilated cardiomyopathy (DCM), death or heart transplantation.7 Traditionally, when the diagnosis was only based upon the histological Dallas diagnostic criteria, myocarditis was considered to be a relatively rare cause of heart failure and/or of sudden cardiac death.8 ,9 Nowadays, the use of highly sensitive immunohistochemical and molecular tools applied to EMB10–18 and of cardiovascular MR (CMR) for non-invasive imaging19–21 suggests that we may be indeed …

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