Cardiac amyloidosis (CA) has emerged as a previously underestimated cause of heart failure and mortality. Underdiagnosis resulted mainly from unawareness of the true disease prevalence and the non-specific symptoms of the disease. CA results from extracellular deposition of misfolded protein fibrils, commonly derived from transthyretin (ATTR) or immunoglobulin light chains (AL). A significant proportion of older patients with heart failure and other extracardiac manifestations suffer from ATTR-CA, whereas AL-CA is still considered a rare disease. This article provides an overview of CA with a special focus on current and emerging diagnostic modalities. Furthermore, we provide a diagnostic algorithm for the evaluation of patients with suspected CA in every-day practice.
Keywords: 99mTc-DPD, 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid; AA, amyloid A amyloidosis; AApoA-1, apolipoprotein A-1 amyloidosis; AL, light chain amyloidosis; ATTR, transthyretin amyloidosis; ATTRv, variant transthyretin amyloidosis; ATTRwt, wild type transthyretin amyloidosis; Amyloidosis; CA, cardiac amyloidosis; Cardiomyopathy; ECV, Extracellular volume; EMB, endomyocardial biopsy; Heart failure; LGE, late gadolinium enhancement; LV, left ventricular/ left ventricular; Light chains; MGUS, monoclonal gammopathy of undetermined significance; MRI, magnetic resonance imaging; NT-proBNP, N-terminal pro B-type natriuretic peptide; PET, positron-emission tomography; SPECT, single photon emission computed tomography; Transthyretin.
© 2020 The Authors.