title: AL (Light-Chain) Cardiac Amyloidosis: A Review of Diagnosis and Therapy.
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.
authors.affiliation: Brigham and Women's Hospital Cardiac Amyloidosis Program, Harvard Medical School and Department of Medicine, Section of Cardiology, Brigham and Women's Hospital, Boston, Massachusetts. Electronic address: rfalk@partners.org.
authors.forename: Rodney H
authors.initials: RH
authors.lastname: Falk
authors.affiliation: Brigham and Women's Hospital Cardiac Amyloidosis Program, Harvard Medical School and Department of Medicine, Section of Cardiology, Brigham and Women's Hospital, Boston, Massachusetts.
authors.forename: Kevin M
authors.initials: KM
authors.lastname: Alexander
authors.affiliation: Brigham and Women's Hospital Cardiac Amyloidosis Program, Harvard Medical School and Department of Medicine, Section of Cardiology, Brigham and Women's Hospital, Boston, Massachusetts.
authors.forename: Ronglih
authors.initials: R
authors.lastname: Liao
authors.affiliation: Brigham and Women's Hospital Cardiac Amyloidosis Program, Harvard Medical School and Department of Medicine, Section of Cardiology, Brigham and Women's Hospital, Boston, Massachusetts; Department of Radiology, Division of Nuclear Medicine and Molecular Imaging, Harvard Medical School, Boston, Massachusetts.
authors.forename: Sharmila
authors.initials: S
authors.lastname: Dorbala
mesh_terms: D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy*; D003663:Decision Trees; D006331:Heart Diseases / Q000175:diagnosis* / Q000628:therapy*; D016027:Heart Transplantation; D006801:Humans
chemical_list: D000682:Amyloid
keywords: TTR amyloidosis; cardiomyopathy; multiple myeloma