title: T1 mapping and survival in systemic light-chain amyloidosis.
abstract: OBJECTIVE
  To assess the prognostic value of myocardial pre-contrast T1 and extracellular volume (ECV) in systemic amyloid light-chain (AL) amyloidosis using cardiovascular magnetic resonance (CMR) T1 mapping.
  
  
  RESULTS
  One hundred patients underwent CMR and T1 mapping pre- and post-contrast. Myocardial ECV was calculated at contrast equilibrium (ECV(i)) and 15 min post-bolus (ECVb). Fifty-four healthy volunteers served as controls. Patients were followed up for a median duration of 23 months and survival analyses were performed. Mean ECV(i) was raised in amyloid (0.44 ± 0.12) as was ECV(b) (mean 0.44 ± 0.12) compared with healthy volunteers (0.25 ± 0.02), P < 0.001. Native pre-contrast T1 was raised in amyloid (mean 1080 ± 87 ms vs. 954 ± 34 ms, P < 0.001). All three correlated with pre-test probability of cardiac involvement, cardiac biomarkers, and systolic and diastolic dysfunction. During follow-up, 25 deaths occurred. An ECV(i) of >0.45 carried a hazard ratio (HR) for death of 3.84 [95% confidence interval (CI): 1.53-9.61], P = 0.004 and pre-contrast T1 of >1044 ms = HR 5.39 (95% CI: 1.24-23.4), P = 0.02. Extracellular volume after primed infusion and ECVb performed similarly. Isolated post-contrast T1 was non-predictive. In Cox regression models, ECV(i) was independently predictive of mortality (HR = 4.41, 95% CI: 1.35-14.4) after adjusting for E:E', ejection fraction, diastolic dysfunction grade, and NT-proBNP.
  
  
  CONCLUSIONS
  Myocardial ECV (bolus or infusion technique) and pre-contrast T1 are biomarkers for cardiac AL amyloid and they predict mortality in systemic amyloidosis.
authors.affiliation: The Heart Hospital, 16-18 Westmoreland Street, London W1G 8PH, UK The National Amyloidosis Centre, Division of Medicine, UCL Medical School, Royal Free Hospital, Rowland Hill Street, London NW3 2PF, UK Institute of Cardiovascular Science, University College London, Gower Street, London WC1E 6BT, UK.
authors.forename: Sanjay M
authors.initials: SM
authors.lastname: Banypersad
authors.affiliation: The Heart Hospital, 16-18 Westmoreland Street, London W1G 8PH, UK The National Amyloidosis Centre, Division of Medicine, UCL Medical School, Royal Free Hospital, Rowland Hill Street, London NW3 2PF, UK.
authors.forename: Marianna
authors.initials: M
authors.lastname: Fontana
authors.affiliation: The Heart Hospital, 16-18 Westmoreland Street, London W1G 8PH, UK.
authors.forename: Viviana
authors.initials: V
authors.lastname: Maestrini
authors.affiliation: The Heart Hospital, 16-18 Westmoreland Street, London W1G 8PH, UK.
authors.forename: Daniel M
authors.initials: DM
authors.lastname: Sado
authors.affiliation: The Heart Hospital, 16-18 Westmoreland Street, London W1G 8PH, UK The National Amyloidosis Centre, Division of Medicine, UCL Medical School, Royal Free Hospital, Rowland Hill Street, London NW3 2PF, UK.
authors.forename: Gabriella
authors.initials: G
authors.lastname: Captur
authors.affiliation: Biostatistics Unit, UCL Eastman Dental Institute, 256 Grays Inn Road, London WC1X 8LD, UK.
authors.forename: Aviva
authors.initials: A
authors.lastname: Petrie
authors.affiliation: Oxford Centre for Clinical Magnetic Resonance Research, Division of Cardiovascular Medicine, Radcliffe Department of Medicine, University of Oxford, Oxford OX3 9DU, UK.
authors.forename: Stefan K
authors.initials: SK
authors.lastname: Piechnik
authors.affiliation: The National Amyloidosis Centre, Division of Medicine, UCL Medical School, Royal Free Hospital, Rowland Hill Street, London NW3 2PF, UK.
authors.forename: Carol J
authors.initials: CJ
authors.lastname: Whelan
authors.affiliation: The Heart Hospital, 16-18 Westmoreland Street, London W1G 8PH, UK.
authors.forename: Anna S
authors.initials: AS
authors.lastname: Herrey
authors.affiliation: The National Amyloidosis Centre, Division of Medicine, UCL Medical School, Royal Free Hospital, Rowland Hill Street, London NW3 2PF, UK.
authors.forename: Julian D
authors.initials: JD
authors.lastname: Gillmore
authors.affiliation: The National Amyloidosis Centre, Division of Medicine, UCL Medical School, Royal Free Hospital, Rowland Hill Street, London NW3 2PF, UK.
authors.forename: Helen J
authors.initials: HJ
authors.lastname: Lachmann
authors.affiliation: The National Amyloidosis Centre, Division of Medicine, UCL Medical School, Royal Free Hospital, Rowland Hill Street, London NW3 2PF, UK.
authors.forename: Ashutosh D
authors.initials: AD
authors.lastname: Wechalekar
authors.affiliation: The National Amyloidosis Centre, Division of Medicine, UCL Medical School, Royal Free Hospital, Rowland Hill Street, London NW3 2PF, UK.
authors.forename: Philip N
authors.initials: PN
authors.lastname: Hawkins
authors.affiliation: The Heart Hospital, 16-18 Westmoreland Street, London W1G 8PH, UK Institute of Cardiovascular Science, University College London, Gower Street, London WC1E 6BT, UK james.moon@uclh.nhs.uk.
authors.forename: James C
authors.initials: JC
authors.lastname: Moon
mesh_terms: D000682:Amyloid / Q000378:metabolism; D000686:Amyloidosis / Q000401:mortality* / Q000473:pathology; D015415:Biomarkers / Q000378:metabolism; D009202:Cardiomyopathies / Q000401:mortality* / Q000473:pathology; D003287:Contrast Media; D005260:Female; D005682:Gadolinium; D006333:Heart Failure / Q000378:metabolism / Q000401:mortality; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism; D053208:Kaplan-Meier Estimate; D018810:Magnetic Resonance Angiography; D008297:Male; D008875:Middle Aged; D011379:Prognosis
chemical_list: D000682:Amyloid; D015415:Biomarkers; D003287:Contrast Media; D007147:Immunoglobulin Light Chains; D005682:Gadolinium
keywords: Amyloid; CMR; Cardiomyopathy; ECV; Heart failure; T1 mapping