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Cardiac biomarkers are prognostic in systemic light chain amyloidosis with no cardiac involvement by standard criteria

Sharpley, FA; Fontana, M; Martinez-Naharro, A; Manwani, R; Mahmood, S; Sachchithanantham, S; Lachmann, HJ; ... Wechalekar, AD; + view all (2019) Cardiac biomarkers are prognostic in systemic light chain amyloidosis with no cardiac involvement by standard criteria. Haematologica 10.3324/haematol.2019.217695. (In press). Green open access

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Abstract

Patients with systemic AL amyloidosis with no evidence of cardiac involvement by consensus criteria have excellent survival, but 20% will die within 5 years of diagnosis and prognostic factors remain poorly characterised. We report the outcomes of 378 prospectively followed Mayo Stage I patients (N-terminal pro b-type natriuretic peptide <332 ng/L, high sensitivity cardiac troponin <55ng/L). The median presenting N-terminal pro b-type natriuretic peptide was 161 ng/L, high sensitivity cardiac troponin 10 ng/L, creatinine 76 μmol/L and mean left ventricular septal wall thickness, 10mm. Median follow up was 42 (1-117 months), with 71 deaths; median overall survival was not reached (78% survival at 5 years). Although no patients had cardiac involvement by echocardiogram, a proportion (N=25/90, 28%) had cardiac involvement by cardiac magnetic resonance imaging. Age, autonomic nervous system involvement, N-terminal pro b-type natriuretic peptide >152 ng/L, high sensitivity cardiac troponin >10 ng/L and cardiac involvement by magnetic resonance imaging were predictive for survival; on multivariate analysis only N-terminal pro b-type natriuretic peptide >152 ng/L (p=<0.008, HR 3.180, CI=1.349-7.495) and cardiac involvement on magnetic resonance imaging (p=0.026, HR=5.360, CI=1.219-23.574) were prognostic. At 5 years, 70% of patients with N-terminal pro b-type natriuretic peptide >152 ng/L were alive. In conclusion, N-terminal pro b-type natriuretic peptide is prognostic for survival in patients with no cardiac involvement by consensus criteria and cardiac involvement is detected by magnetic resonance imaging in such cases. This suggests that N-terminal pro b-type natriuretic peptide thresholds for cardiac involvement in AL amyloidosis may need to be redefined.

Type: Article
Title: Cardiac biomarkers are prognostic in systemic light chain amyloidosis with no cardiac involvement by standard criteria
Location: Italy
Open access status: An open access version is available from UCL Discovery
DOI: 10.3324/haematol.2019.217695
Publisher version: https://doi.org/10.3324/haematol.2019.217695
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Amyloidosis, Cardiac magnetic resonance imaging, Prognostic factors
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Inflammation
URI: https://discovery.ucl.ac.uk/id/eprint/10080185
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