Imaging Options in Cardiac Amyloidosis: Differentiating AL from ATTR

AbstractPurpose of ReviewCardiac amyloidosis (CA) results from the myocardial deposition of abnormally folded endogenous proteins leading to significant morbidity and mortality. Accurate and timely non-invasive diagnosis of cardiac amyloidosis and the correct identification of amyloid type are critical for choosing appropriate therapy. Disease type is defined by the precursor protein and most typically involves light chain (AL) or transthyretin (ATTR). Traditionally, cardiac amyloidosis has been suspected on the basis of characteristic echocardiographic features and confirmed with endomyocardial biopsy. Recent advances in echocardiography, cardiovascular magnetic resonance (CMR), and nuclear imaging now permit the non-invasive diagnosis of CA with a high degree of confidence and, in some cases, assignment of amyloid types.Recent FindingsCMR techniques including late gadolinium enhancement and new protocols such as T1 mapping and extra-cellular volume (ECV) quantification have afforded insight into the diagnosis and pathophysiology of cardiac amyloidosis, while bone-avid nuclear tracers can identify ATTR with a high degree of accuracy.SummaryWe highlight the recent advances in imaging techniques that can identify cardiac amyloidosis with a special emphasis on nuclear modalities used for differentiating light chain from transthyretin disease.

Source: Current Cardiovascular Imaging Reports - January 31, 2017 Category: Radiology Source Type: research