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Amyloidosis cardiomyopathy: update in the diagnosis and treatment of the most common types

Nativi-Nicolau, Josea; Maurer, Mathew S.b

doi: 10.1097/HCO.0000000000000547
Special Commentary

Purpose of review The present article provides an update about the recent advances in the diagnosis and management of the most common types of cardiac amyloidosis, including light chain, wild-type transthyretin (ATTRwt), and mutant transthyretin (ATTRm).

Recent findings The document reviews the utility of diagnostic tools including innovative echocardiographic indices, magnetic resonance T1 mapping and measurement of extracellular volume, and the role and validation of bone scintigraphy for the noninvasive assessment of ATTR amyloidosis. It summarizes the data about therapies for light chain amyloidosis including bortezomib regimens and also novel disease modifying therapies for ATTR amyloidosis such as gene silencers, transthyretin stabilizers, and degraders of amyloid fibrils.

Summary The present review provides the readers with the necessary tools in order to recognize and diagnose cardiac amyloidosis early and introduces the recent advances in management that are improving the outcomes of a condition that was considered to be untreatable.

aUniversity of Utah, Salt Lake City, Utah

bColumbia University Medical Center, New York, New York, USA

Correspondence to Jose Nativi-Nicolau, Assistant Professor of Medicine, Director, Cardiac Amyloidosis Program, University of Utah, 50 N Medical Dr, Salt Lake City, UT 84132, USA. Tel: +801 585 2343; fax: +801 581 7735; e-mail: jose.nativi-nicolau@hsc.utah.edu

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