Giant cell myocarditis in modern clinical practice

Abstract

Giant cell myocarditis is a rare but extremely severe disease with a frequent need for heart transplantation and a high mortality rate. To this day, the most common reason for the occurrence of this disease has not been precisely determined, but its frequent association with autoimmune diseases speaks in favor of autoimmune etiology. Clinically, it is presented to the greatest extent by symptoms and signs of acute heart failure and ventricular rhythm disturbances, which are registered in as many as half of the patients. Arrhythmias can be accompanied by the occurrence of palpitations, repeated syncope, and even sudden cardiac death. A severe degree of acute heart failure in hemodynamically unstable patents who respond inadequately to the administered therapy for heart failure and refractory heart rhythm disorders and/or conduction disorders that are common in these patients should always raise suspicion of giant cell myocarditis. Given that changes in electrocardiogram, echocardiography and positive biomarker values are not strictly specific for this disease, the diagnosis of giant cell myocarditis is most often established by endomyocardial biopsy, especially in patients with the fulminant form of the disease. Timely endomyocardial biopsy enables not only quick and accurate diagnosis, but also early administration of immunosuppressive therapy, which greatly improves the outcome in these patients. Pathohistological verification of this disease is important so as to rule out cardiac sarcoidosis and other granulomatous infectious and non-infectious diseases. Given that in a large number of patients the disease has a fulminant course, early and rapid diagnosis, application of inotropic stimulation and mechanical circulatory support in hemodynamically unstable patients and adequate modern therapeutic regimen can largely reduce mortality in these patients, which is still high despite modern diagnostics and therapeutic progress of medicine.