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AL (Light-Chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy

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source: Journal of the American College of Cardiology

year: 2016

authors: Falk RH, Alexander KM, Liao R, Dorbala S

summary/abstract:

The amyloidoses are a group of protein-folding disorders in which >=1 organ is infiltrated by proteinaceous deposits known as amyloid. The deposits are derived from 1 of several amyloidogenic precursor proteins, and the prognosis of the disease is determined both by the organ(s) involved and the type of amyloid. 

Amyloid involvement of the heart (cardiac amyloidosis) carries the worst prognosis of any involved organ, and light-chain (AL) amyloidosis is the most serious form of the disease. The last decade has seen considerable progress in understanding the amyloidoses. In this review, current and novel approaches to the diagnosis and treatment of cardiac amyloidosis are discussed, with particular reference to AL amyloidosis in the heart.

organization: Harvard Medical School and Department of Medicine, USA; Brigham and Women's Hospital, USA

DOI: 10.1016/j.jacc.2016.06.053

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