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Myocarditis in systemic inflammatory diseases

Myocardial Disease

Rheumatic heart disease

Dr Ganesan Karthikeyan (India) reported about rheumatic heart disease, which remains a major cause of morbidity and mortality in developing countries. Rheumatic carditis mainly involves the endocardium (heart valves) and occurs in up to 90% of patients with acute rheumatic fever, often leading to chronic rheumatic heart disease. Currently, there is no treatment of any proven efficacy for acute rheumatic fever and carditis. Primary and secondary prevention via the use of antibiotics and polyvalent vaccines against group A hemolytic streptococci offer the only means to reduce the burden of disease.

Giant cell and eosinophilic myocarditis

Dr. Alida Caforio (Italy) summarized the state of the art regarding idiopathic giant cell myocarditis (GCM) and eosinophilic myocarditis, which both may present as life-threatening diseases. Prompt diagnosis of GCM is essential for the patient's survival, which can be achieved by aggressive medical therapy including mechanical assist device and immunosuppression. Eosinophilic myocarditis has a heterogeneous etiology including idiopathic, infectious, hypersensitivity, immune-mediated and hematological (myeloproliferative) causes. The management of patients with GCM and eosinophilic myocarditis needs a careful diagnostic work-up by expert multidisciplinary teams, since prognosis depends on endomyocardial biopsy-confirmed and molecular etiologic diagnosis and etiology-driven therapy (see: Caforio et al., Eur Heart J. 2013 Jul 3. [Epub ahead of print]).


Dr. Leslie Cooper (USA) provided expert guidance on cardiac sarcoidosis (CS), which often presents as a chronic, progressive cardiomyopathy associated with high grade heart block and/or ventricular arrhythmias. The diagnosis is based on a compatible clinical syndrome, cardiac MRI and/or FDG/Ammonia PET scan and endomyocardial biopsy (EMB). Without electrophysiological mapping catheter guidance, the sensitivity of right ventricular EMB for CS is between 20% and 30%. Treatment consists of moderate to high dose corticosteroids, sometimes with a steroid sparing agent like azathioprine or mycophenolate mofetil to minimize steroid-related side effects. The 5 year combined risk of heart transplantation and death is higher in CS than in lymphocytic myocarditis or non-ischemic cardiomyopathy.

Connective tissue diseases

Dr. Antonio Brucato (Italy) summarized findings in patients with myocarditis related to connective tissue diseases. He reported that in mainly ANCA negative patients, 8-20% of the cases with Churg-Strauss syndrome, now renamed eosinophilic granulomatosis with polyangiitis (EGPA), the heart is involved. He described a series of 9 Churg-Strauss patients who had to undergo heart transplantation (Tx). Delay in diagnosis was common, and eosinophilia was occasionally absent. Clinical and histological activity was severe at Tx. Post Tx standard immunosuppressive therapy was often unable to control EGPA recurrence, both systemic and cardiac. Four patients died, all suddenly, thus these patients should be considered as high-risk patients for arrhythmias.


Session Title: Myocarditis in systemic inflammatory diseases

The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.