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Light-chain cardiac amyloidosis: strategies to promote early diagnosis and cardiac response.
Heart. 2017 07; 103(14):1065-1072.H

Abstract

Amyloid light chain (AL) amyloidosis is a systemic disease characterised by the aggregation of misfolded immunoglobulin light chain (LC), predominantly in the heart and kidneys, causing organ failure. If untreated, the median survival of patients with cardiac AL amyloidosis is 6 months from the onset of heart failure. Protracted time to establish a diagnosis, often lasting >1 year, is a frequent factor in poor treatment outcomes. Cardiologists, to whom patients are often referred, frequently miss the opportunity to diagnose cardiac AL amyloidosis. Nearly all typical cardiac support measures, with the exception of diuretics, are ineffective and may even worsen clinical symptoms, emphasising the need for accurate diagnosis. Patients with severe cardiac involvement face poor outcomes; heart transplantation is rarely an option because of multiorgan involvement, rapid clinical decline and challenges in predicting which patients will respond to treatment of the underlying plasma cell disorder. Early diagnosis and prompt treatment with ââ'¬Ëœsource therapiesââ'¬â"¢ that limit the production of amyloidogenic LC are associated with better survival and improvement in organ function after a median of 2.4 months following haematological complete response. However, organ recovery is often incomplete because these source therapies do not directly target deposited amyloid. Emerging amyloid-directed therapies may attenuate, and potentially reverse, organ dysfunction by clearing existing amyloid and inhibiting fibril formation of circulating aggregates. Improved recognition of AL amyloidosis by cardiologists allows for earlier treatment and improved outcomes.

Authors+Show Affiliations

Division of Cardiovascular Diseases, Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA.Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA.Division of Hematology, Department of Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

28456755

Citation

Grogan, Martha, et al. "Light-chain Cardiac Amyloidosis: Strategies to Promote Early Diagnosis and Cardiac Response." Heart (British Cardiac Society), vol. 103, no. 14, 2017, pp. 1065-1072.
Grogan M, Dispenzieri A, Gertz MA. Light-chain cardiac amyloidosis: strategies to promote early diagnosis and cardiac response. Heart. 2017;103(14):1065-1072.
Grogan, M., Dispenzieri, A., & Gertz, M. A. (2017). Light-chain cardiac amyloidosis: strategies to promote early diagnosis and cardiac response. Heart (British Cardiac Society), 103(14), 1065-1072. https://doi.org/10.1136/heartjnl-2016-310704
Grogan M, Dispenzieri A, Gertz MA. Light-chain Cardiac Amyloidosis: Strategies to Promote Early Diagnosis and Cardiac Response. Heart. 2017;103(14):1065-1072. PubMed PMID: 28456755.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Light-chain cardiac amyloidosis: strategies to promote early diagnosis and cardiac response. AU - Grogan,Martha, AU - Dispenzieri,Angela, AU - Gertz,Morie A, Y1 - 2017/04/29/ PY - 2016/09/29/received PY - 2017/01/10/revised PY - 2017/02/04/accepted PY - 2017/5/1/pubmed PY - 2017/8/10/medline PY - 2017/5/1/entrez KW - Cardiac imaging and diagnostics KW - Infiltrative cardiomyopathies SP - 1065 EP - 1072 JF - Heart (British Cardiac Society) JO - Heart VL - 103 IS - 14 N2 - Amyloid light chain (AL) amyloidosis is a systemic disease characterised by the aggregation of misfolded immunoglobulin light chain (LC), predominantly in the heart and kidneys, causing organ failure. If untreated, the median survival of patients with cardiac AL amyloidosis is 6 months from the onset of heart failure. Protracted time to establish a diagnosis, often lasting >1 year, is a frequent factor in poor treatment outcomes. Cardiologists, to whom patients are often referred, frequently miss the opportunity to diagnose cardiac AL amyloidosis. Nearly all typical cardiac support measures, with the exception of diuretics, are ineffective and may even worsen clinical symptoms, emphasising the need for accurate diagnosis. Patients with severe cardiac involvement face poor outcomes; heart transplantation is rarely an option because of multiorgan involvement, rapid clinical decline and challenges in predicting which patients will respond to treatment of the underlying plasma cell disorder. Early diagnosis and prompt treatment with ââ'¬Ëœsource therapiesââ'¬â"¢ that limit the production of amyloidogenic LC are associated with better survival and improvement in organ function after a median of 2.4 months following haematological complete response. However, organ recovery is often incomplete because these source therapies do not directly target deposited amyloid. Emerging amyloid-directed therapies may attenuate, and potentially reverse, organ dysfunction by clearing existing amyloid and inhibiting fibril formation of circulating aggregates. Improved recognition of AL amyloidosis by cardiologists allows for earlier treatment and improved outcomes. SN - 1468-201X UR - https://www.unboundmedicine.com/medline/citation/28456755/Light_chain_cardiac_amyloidosis:_strategies_to_promote_early_diagnosis_and_cardiac_response_ L2 - http://heart.bmj.com/cgi/pmidlookup?view=long&pmid=28456755 DB - PRIME DP - Unbound Medicine ER -