Diagnostics and treatment of cardiac amyloidosis: Position paper of the German Cardiac Society (DGK)

Abstract

Systemic forms of amyloidosis that are associated with cardiac involvement are in particular light-chain (AL) and transthyretin (ATTR) amyloidosis, which is caused by deposition of incorrectly folded transthyretin, either as the wild-type (wtATTR) or mutated (mATTR) form. Apart from specific cardiac biomarkers, modern noninvasive imaging procedures, such as cardiovascular magnetic resonance imaging (CMRI) and scintigraphic methods are available today. These imaging procedures not only complement conventional echocardiography but also enable accurate assessment of the extent of cardiac involvement in addition to the diagnosis of cardiac amyloidosis. In addition, invasive endomyocardial biopsy still plays a major role in histopathological confirmation of the underlying diagnosis and subsequent subtyping of cardiac amyloidosis. The major goal of the diagnostic algorithm illustrated in the present position paper is a definitive and timely diagnosis of the presence of cardiac amyloidosis, to accurately assess the extent of cardiac involvement, to quickly and safely identify the underlying subtype of amyloidosis and to subsequently enable a targeted treatment.