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Measurement of myocardial amyloid deposition in systemic amyloidosis: insights from cardiovascular magnetic resonance imaging.
MedLine Citation:
PMID:  25346163     Owner:  NLM     Status:  Publisher    
BACKGROUND: Cardiac involvement in systemic amyloidosis is caused by the extracellular deposition of misfolded proteins, mainly immunoglobulin light chains (AL) or transthyretin (ATTR), and may be detected by cardiovascular magnetic resonance (CMR). The aim of this study was to measure myocardial extracellular volume (ECV) in amyloid patients with a novel T1 mapping CMR technique and to determine the correlation between ECV and disease severity.
METHODS: Thirty-six patients with biopsy-proven systemic amyloidosis (mean age 70±9 years, 31 men, 30 with AL and six with ATTR amyloidosis) and seven patients with possible amyloidosis (mean age 64±10 years, six men) underwent comprehensive clinical and CMR assessment, with ECV estimation from pre- and post-contrast T1 mapping. Thirty healthy subjects (mean age 39±17 years, 21 men) served as the control group.
RESULTS: Amyloid patients presented with left ventricular (LV) concentric hypertrophy with impaired biventricular systolic function. Cardiac ECV was higher in amyloid patients (definite amyloidosis, 0.43±0.12; possible amyloidosis, 0.34±0.11) than in control subjects (0.26±0.04, P < 0.05); even in amyloid patients without late gadolinium enhancement (0.35±0.10), ECV was significantly higher than in the control group (P < 0.01). A cut-off value of myocardial ECV >0.316, corresponding to the 95(th) percentile in normal subjects, showed a sensitivity of 79% and specificity of 97% for discriminating amyloid patients from control subjects (area under the curve of 0.884). Myocardial ECV was significantly correlated with LV ejection fraction (R(2) =0.16), LV mean wall thickness (R(2) =0.41), LV diastolic function (R(2) =0.21), right ventricular ejection fraction (R(2) =0.13), N-terminal fragment of the pro-brain natriuretic peptides (R(2) =0.23) and cardiac troponin (R(2) =0.33).
CONCLUSION: Myocardial ECV was increased in amyloid patients and correlated with disease severity. Thus measurement of myocardial ECV represents a potential non-invasive index of amyloid burden for use in early diagnosis and disease monitoring. This article is protected by copyright. All rights reserved.
Andrea Barison; Giovanni Donato Aquaro; Nicola Riccardo Pugliese; Francesco Cappelli; Sara Chiappino; Giuseppe Vergaro; Gianluca Mirizzi; Giancarlo Todiere; Claudio Passino; Pier Giorgio Masci; Federico Perfetto; Michele Emdin
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-10-24
Journal Detail:
Title:  Journal of internal medicine     Volume:  -     ISSN:  1365-2796     ISO Abbreviation:  J. Intern. Med.     Publication Date:  2014 Oct 
Date Detail:
Created Date:  2014-10-27     Completed Date:  -     Revised Date:  2014-10-28    
Medline Journal Info:
Nlm Unique ID:  8904841     Medline TA:  J Intern Med     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
This article is protected by copyright. All rights reserved.
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