Myocardial Involvement in Systemic Immune-Mediated Diseases

Caforio AL, Adler Y, Agostini C, et al.
Diagnosis and Management of Myocardial Involvement in Systemic Immune-Mediated Diseases: A Position Statement of the European Society of Cardiology Working Group on Myocardial and Pericardial Disease. Eur Heart J 2017;Jun 26:[Epub ahead of print].

The following are key points to remember about this European Society of Cardiology (ESC) position statement on the diagnosis and management of myocardial involvement in systemic immune-mediated diseases (SIDs):

  1. SIDs include autoimmune and autoinflammatory diseases affecting at least two-organ systems. Cardiac involvement in SIDs is associated with adverse outcomes.
  2. Currently there is a lack of up to date cardiological diagnostic workup in the scientific literature and in clinical practice, leading to poor scientific knowledge, late recognition, or underdiagnosis and undertreatment of cardiac involvement.
  3. Clinical presentation of myocarditis is unspecific. Red flags may include: unexplained dyspnea, palpitations, chest pain with or without increased troponin, syncope, arrhythmia and acute or chronic congestive heart failure, aborted sudden cardiac death, and fulminant cardiogenic shock.
  4. Since in many SIDs accelerated coronary artery disease as well as coronary microvascular dysfunction may be predominant or contributory to cardiac signs and symptoms, an ischemic etiology should be ruled out first, whenever clinically indicated, by standard noninvasive and invasive means.
  5. The specific workup should be tailored to the individual case and clinically oriented.
  6. Endomyocardial biopsy (EMB) is the gold standard for suspected myocarditis with or without associated SIDs; using current histological, immunological, histochemical, and molecular tools, it provides differentiation between infectious and noninfectious myocarditis.
  7. Ideally and whenever possible, sophisticated and expensive second-step cardiac tests (e.g., cardiac magnetic resonance, positron emission tomography, and EMB) should be performed in specialized centers, experienced in rare cardiac disease assessment.
  8. EMB may foster treatment decisions on the basis of histopathological results, particularly in eosinophilic myocarditis, sarcoidosis, and giant cell myocarditis. In these types of noninfectious myocarditis, immunosuppressive therapy should always be considered, provided that major contraindications are excluded (e.g., active or latent malignancy or extracardiac infection).
  9. The use of biomarkers such as natriuretic peptides should be adapted according to current ESC heart failure guidelines, algorithms, and cut-off values. It should not be done routinely, but only if clinical suspicion of cardiac symptoms and/or signs such as dyspnea develop.
  10. At present, there is a relative paucity of evidence-based treat-to-target regimens in SIDs patients with myocardial involvement besides empirically driven intensification of conventional immunosuppression.

Clinical Topics: Arrhythmias and Clinical EP, Heart Failure and Cardiomyopathies, Noninvasive Imaging, Atherosclerotic Disease (CAD/PAD), Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Acute Heart Failure, Heart Failure and Cardiac Biomarkers

Keywords: Arrhythmias, Cardiac, Biological Markers, Biopsy, Cardiac Imaging Techniques, Chest Pain, Coronary Artery Disease, Death, Sudden, Cardiac, Dyspnea, Heart Failure, Immunosuppression, Magnetic Resonance Spectroscopy, Myocarditis, Myocardium, Natriuretic Peptides, Sarcoidosis, Shock, Cardiogenic, Syncope, Troponin

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