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Native T1 mapping in ATTR cardiac amyloidosis - comparison with AL cardiac amyloidosis - a 200 patient study
© Fontana et al.; licensee BioMed Central Ltd. 2014
- Published: 16 January 2014
- Mutation Carrier
- Cardiac Amyloidosis
- ATTR Amyloidosis
- Transthyretin Amyloidosis
Transthyretin amyloidosis (ATTR amyloidosis) is an under diagnosed cause of heart failure with no truly quantitative test. Cardiac involvement is the leading cause of death and influences therapeutic choices. Since new therapies are imminent which aim to treat ATTR amyloidosis the lack of a quantitative test represents a critical step for drug development. In cardiac AL amyloidosis, T1 has high diagnostic accuracy and tracks disease. We hypothesised similar results would occur for ATTR. We hypothesized that the native myocardial T1 would be elevated in ATTR amyloid; that T1 elevation would track the cardiac amyloid burden as measured by DPD grading; and that T1 elevation would be an early disease marker.
3 groups were studied: ATTR amyloid patients (n = 85; 70 male; age 73 ± 10); healthy mutations carriers (n = 8; 3 male; age 47 ± 6); and AL amyloid patients (n = 79; 55 male; age 62 ± 10). These were compared with 52 healthy volunteers and 46 patients with hypertrophic cardiomyopathy (HCM). All underwent T1 mapping (Shortened Modified Look-Locker Inversion recovery, ShMOLLI). ATTR patients and mutation carriers also underwent cardiac DPD scintigraphy.
Native myocardial T1 mapping represents the first test able in ATTR amyloidosis to quantify the cardiac amyloid burden. Native myocardial T1 detects cardiac ATTR amyloid with similar diagnostic performance and potential for quantitation as AL amyloid, but with lower maximal T1 elevation and appears to be an early marker of disease.
Dr Fontana Is funded by the British Heart Foundation. A proportion of the scans have been funded by GlaxoSmithKline.
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.