Clinical characteristics and prognosis of cardiac amyloidosis defined by mass spectrometry-based proteomics in an Australian cohort.
- Publisher:
- Wiley
- Publication Type:
- Journal Article
- Citation:
- Internal medicine journal, 2020
- Issue Date:
- 2020-09-27
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Cardiac amyloidosis has a very poor prognosis, but it is the nature of the involved precursor protein that ultimately dictates treatment and survival. We report the clinical characteristics and survival of 47 cardiac amyloid patients across 2 Australian centres including 39 patients evaluated for definitive amyloid subtype utilising laser microdissection and tandem mass spectrometry (LMD-MS). A quarter of patients (n=12) were classified as wild type transthyretin amyloidosis (ATTRwt), 33 patients as light or heavy chain amyloidosis (AL or AH), and 2 as hereditary mutant transthyretin amyloidosis (ATTRv). Greater left ventricular hypertrophy (IV septum 22 vs. 15 mm, p=0.005) and history of cardiac arrhythmia (75% vs. 31%, p=0.016) were significantly associated with ATTRwt patients compared with AL/AH patients. AL patients demonstrated significantly shorter median survival compared to ATTRwt patients (3.5 vs. 37 months, (P=0.007)). New York heart association (NYHA) class III-IV symptoms or plasma cells ≥ 10% at diagnosis, were the only independent predictors of worse survival in AL patients on multivariate analysis. In the era of novel therapies for both AL amyloid and ATTR, identification of the correct amyloid subtype is essential in making therapeutic decisions and providing accurate prognostic information to patients. This article is protected by copyright. All rights reserved.
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