4.8 Article

Addressing Common Questions Encountered in the Diagnosis and Management of Cardiac Amyloidosis

Journal

CIRCULATION
Volume 135, Issue 14, Pages 1357-1377

Publisher

LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1161/CIRCULATIONAHA.116.024438

Keywords

amyloidosis; cardiomyopathies; echocardiography; heart failure; diastolic; immunoglobulin light chains; magnetic resonance imaging; prealbumin; radionuclide imaging

Funding

  1. National Institute on Aging [AG036778-06]

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Advances in cardiac imaging have resulted in greater recognition of cardiac amyloidosis in everyday clinical practice, but the diagnosis continues to be made in patients with late-stage disease, suggesting that more needs to be done to improve awareness of its clinical manifestations and the potential of therapeutic intervention to improve prognosis. Light chain cardiac amyloidosis, in particular, if recognized early and treated with targeted plasma cell therapy, can be managed very effectively. For patients with transthyretin amyloidosis, there are numerous therapies that are currently in late-phase clinical trials. In this review, we address common questions encountered in clinical practice regarding etiology, clinical presentation, diagnosis, and management of cardiac amyloidosis, focusing on recent important developments in cardiac imaging and biochemical diagnosis. The aim is to show how a systematic approach to the evaluation of suspected cardiac amyloidosis can impact the prognosis of patients in the modern era.

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