title: CMR imaging with rapid visual T1 assessment predicts mortality in patients suspected of cardiac amyloidosis.
abstract: OBJECTIVE
  This study tested the diagnostic and prognostic utility of a rapid, visual T1 assessment method for identification of cardiac amyloidosis (CA) in a "real-life" referral population undergoing cardiac magnetic resonance for suspected CA.
  
  
  BACKGROUND
  In patients with confirmed CA, delayed-enhancement cardiac magnetic resonance (DE-CMR) frequently shows a diffuse, global hyperenhancement (HE) pattern. However, imaging is often technically challenging, and the prognostic significance of diffuse HE is unclear.
  
  
  METHODS
  Ninety consecutive patients referred for suspected CA and 64 hypertensive patients with left ventricular hypertrophy (LVH) were prospectively enrolled and underwent a modified DE-CMR protocol. After gadolinium administration a method for rapid, visual T1 assessment was used to identify the presence of diffuse HE during the scan, allowing immediate optimization of settings for the conventional DE-CMR that followed. The primary endpoint was all-cause mortality.
  
  
  RESULTS
  Among patients with suspected CA, 66% (59 of 90) demonstrated HE, with 81% (48 of 59) of these meeting pre-specified visual T1 assessment criteria for diffuse HE. Among hypertensive LVH patients, 6% (4 of 64) had HE, with none having diffuse HE. During 29 months of follow-up (interquartile range: 12 to 44 months), there were 50 (56%) deaths in patients with suspected CA and 4 (6%) in patients with hypertensive LVH. Multivariable analysis demonstrated that the presence of diffuse HE was the most important predictor of death in the group with suspected CA (hazard ratio: 5.5, 95% confidence interval: 2.7 to 11.0; p < 0.0001) and in the population as a whole (hazard ratio: 6.0, 95% confidence interval 3.0 to 12.1; p < 0.0001). Among 25 patients with myocardial histology obtained during follow-up, the sensitivity, specificity, and accuracy of diffuse HE in the diagnosis of CA were 93%, 70%, and 84%, respectively.
  
  
  CONCLUSIONS
  Among patients suspected of CA, the presence of diffuse HE by visual T1 assessment accurately identifies patients with histologically-proven CA and is a strong predictor of mortality.
authors.affiliation: London Health Sciences Center, Division of Cardiology, University of Western Ontario, London, Ontario, Canada; Robarts Research Institute, University of Western Ontario, London, Ontario, Canada; Lawson Health Research Institute, University of Western Ontario, London, Ontario, Canada.
authors.forename: James A
authors.initials: JA
authors.lastname: White
authors.affiliation: Duke Cardiovascular Magnetic Resonance Center, Duke University Medical Center, Durham, North Carolina; Division of Cardiology, Duke University Medical Center, Durham, North Carolina.
authors.forename: Han W
authors.initials: HW
authors.lastname: Kim
authors.affiliation: The Methodist DeBakey Heart Center, Houston, Texas.
authors.forename: Dipan
authors.initials: D
authors.lastname: Shah
authors.affiliation: London Health Sciences Center, Division of Cardiology, University of Western Ontario, London, Ontario, Canada.
authors.forename: Nowell
authors.initials: N
authors.lastname: Fine
authors.affiliation: Duke Cardiovascular Magnetic Resonance Center, Duke University Medical Center, Durham, North Carolina.
authors.forename: Ki-Young
authors.initials: KY
authors.lastname: Kim
authors.affiliation: Duke Cardiovascular Magnetic Resonance Center, Duke University Medical Center, Durham, North Carolina.
authors.forename: David C
authors.initials: DC
authors.lastname: Wendell
authors.affiliation: Duke Cardiovascular Magnetic Resonance Center, Duke University Medical Center, Durham, North Carolina.
authors.forename: Wael
authors.initials: W
authors.lastname: Al-Jaroudi
authors.affiliation: Duke Cardiovascular Magnetic Resonance Center, Duke University Medical Center, Durham, North Carolina; Division of Cardiology, Duke University Medical Center, Durham, North Carolina.
authors.forename: Michele
authors.initials: M
authors.lastname: Parker
authors.affiliation: Division of Cardiology, Duke University Medical Center, Durham, North Carolina.
authors.forename: Manesh
authors.initials: M
authors.lastname: Patel
authors.affiliation: Lawson Health Research Institute, University of Western Ontario, London, Ontario, Canada.
authors.forename: Femida
authors.initials: F
authors.lastname: Gwadry-Sridhar
authors.affiliation: Duke Cardiovascular Magnetic Resonance Center, Duke University Medical Center, Durham, North Carolina; Division of Cardiology, Duke University Medical Center, Durham, North Carolina; Department of Radiology, Duke University Medical Center, Durham, North Carolina.
authors.forename: Robert M
authors.initials: RM
authors.lastname: Judd
authors.affiliation: Duke Cardiovascular Magnetic Resonance Center, Duke University Medical Center, Durham, North Carolina; Division of Cardiology, Duke University Medical Center, Durham, North Carolina; Department of Radiology, Duke University Medical Center, Durham, North Carolina. Electronic address: raymond.kim@duke.edu.
authors.forename: Raymond J
authors.initials: RJ
authors.lastname: Kim
mesh_terms: D000686:Amyloidosis / Q000175:diagnosis*; D005260:Female; D006331:Heart Diseases / Q000175:diagnosis*; D006801:Humans; D008297:Male
keywords: amyloid; delayed enhancement; magnetic resonance imaging