Document Detail

Evaluation and management of the cardiac amyloidosis.
MedLine Citation:
PMID:  18036445     Owner:  NLM     Status:  MEDLINE    
Cardiac amyloidosis describes clinically significant involvement of the heart by amyloid deposition, which may or may not be associated with involvement of other organs. The purpose of this review is to summarize the current state of evidence for the effective evaluation and management of cardiac amyloidosis. Acquired systemic amyloidosis occurs in more than 10 per million person-years in the U.S. population. Although no single noninvasive test or abnormality is pathognomonic of cardiac amyloid, case-control studies indicate that echocardiographic evidence of left ventricular wall thickening, biatrial enlargement, and increased echogenicity in conjunction with reduced electrocardiographic voltages is strongly suggestive of cardiac amyloidosis. Furthermore, newer echocardiographic techniques such as strain and strain rate imaging can demonstrate impairment in longitudinal function before ejection fraction becomes abnormal. Recent observational studies also suggest that cardiovascular magnetic resonance imaging yields characteristic findings in amyloidosis, offering promise for the early detection of cardiac involvement, and the presence of detectable cardiac troponin and elevated B-type natriuretic peptide in serum of affected patients portends an adverse prognosis. Management strategies for cardiac amyloid are largely based on nonrandomized single-center studies. One of the few published randomized studies shows the superiority of oral prednisolone and melphalan compared with colchicine in systemic AL amyloidosis. Intermediate-dose infusional chemotherapy regimes (such as vincristine, adriamycin, and dexamethasone) and high-dose chemotherapy with peripheral stem cell rescue have been used widely, but treatment-related mortality remains substantial with chemotherapy. Recent studies also indicate promising strategies to stabilize the native structures of amyloidogenic proteins; inhibit fibril formation; and disrupt established deposits using antibodies, synthetic peptides, and small-molecule drugs.
Joseph B Selvanayagam; Philip N Hawkins; Biju Paul; Saul G Myerson; Stefan Neubauer
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review     Date:  2007-11-13
Journal Detail:
Title:  Journal of the American College of Cardiology     Volume:  50     ISSN:  1558-3597     ISO Abbreviation:  J. Am. Coll. Cardiol.     Publication Date:  2007 Nov 
Date Detail:
Created Date:  2007-11-26     Completed Date:  2008-01-07     Revised Date:  2014-02-19    
Medline Journal Info:
Nlm Unique ID:  8301365     Medline TA:  J Am Coll Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2101-10     Citation Subset:  AIM; IM    
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MeSH Terms
Amyloid / blood
Amyloidosis / blood,  diagnosis*,  drug therapy*
Antineoplastic Agents / therapeutic use*
Biological Markers / blood
Heart Diseases / blood,  diagnosis*,  drug therapy*
Magnetic Resonance Imaging
Troponin / blood
Grant Support
G7900510//Medical Research Council
Reg. No./Substance:
0/Amyloid; 0/Antineoplastic Agents; 0/Biological Markers; 0/Troponin
Comment In:
J Am Coll Cardiol. 2008 Apr 15;51(15):1509-10; author reply 1510   [PMID:  18402909 ]
Erratum In:
J Am Coll Cardiol. 2011 Mar 29;57(13):1501

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