Diagnosis and treatment of cardiac amyloidosis A position statement of the ESC working group on myocardial and pericardial diseases 2021

Cardiac amyloidosis is still considered a rare disease, although recent data show that it is the cause of cardiac dysfunction more frequently than expected. The diagnosis of cardiac amyloidosis is based on the detection of extracellular deposits of misfolded proteins in the myocardium. This detection can be made invasively or noninvasively and is based on a tentative diagnosis that forms the foundation for further diagnostic measures. As different forms of amyloidosis may have different clinical presentations, suspicion of amyloidosis is often difficult. As not only the diagnostic possibilities have become broader but also new therapeutic possibilities have been tested in clinical studies, the working group on myocardial and pericardial diseases of the European Society of Cardiology (ESC) has set up a working group of experts to compile the current data on the clinical presentation, diagnostics and treatment of patients with cardiac amyloidosis, in order to subsequently develop diagnostic criteria and treatment options for patients with different forms of cardiac amyloidosis by consensus. The aim was to formulate a uniform Europe-wide acceptable concept for essential diagnostics and treatment for this group of patients. Only this will create the foundation for national and international registers and double-blind randomized treatment studies.