James A White1, Han W Kim2, Dipan Shah3, Nowell Fine4, Ki-Young Kim5, David C Wendell5, Wael Al-Jaroudi5, Michele Parker2, Manesh Patel6, Femida Gwadry-Sridhar7, Robert M Judd8, Raymond J Kim9. 1. 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. 2. Duke Cardiovascular Magnetic Resonance Center, Duke University Medical Center, Durham, North Carolina; Division of Cardiology, Duke University Medical Center, Durham, North Carolina. 3. The Methodist DeBakey Heart Center, Houston, Texas. 4. London Health Sciences Center, Division of Cardiology, University of Western Ontario, London, Ontario, Canada. 5. Duke Cardiovascular Magnetic Resonance Center, Duke University Medical Center, Durham, North Carolina. 6. Division of Cardiology, Duke University Medical Center, Durham, North Carolina. 7. Lawson Health Research Institute, University of Western Ontario, London, Ontario, Canada. 8. 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. 9. 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.
Abstract
OBJECTIVES: 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.
OBJECTIVES: 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 hypertensivepatients 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 LVHpatients, 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: Alicia Maria Maceira; Jayshree Joshi; Sanjay Kumar Prasad; James Charles Moon; Enrica Perugini; Idris Harding; Mary Noelle Sheppard; Philip Alexander Poole-Wilson; Philip Nigel Hawkins; Dudley John Pennell Journal: Circulation Date: 2005-01-03 Impact factor: 29.690
Authors: R J Kim; E Wu; A Rafael; E L Chen; M A Parker; O Simonetti; F J Klocke; R O Bonow; R M Judd Journal: N Engl J Med Date: 2000-11-16 Impact factor: 91.245
Authors: Joseph E Rahman; Emelie F Helou; Ramona Gelzer-Bell; Richard E Thompson; Chih Kuo; E Rene Rodriguez; Joshua M Hare; Kenneth L Baughman; Edward K Kasper Journal: J Am Coll Cardiol Date: 2004-02-04 Impact factor: 24.094
Authors: Sharmila Dorbala; Yukio Ando; Sabahat Bokhari; Angela Dispenzieri; Rodney H Falk; Victor A Ferrari; Marianna Fontana; Olivier Gheysens; Julian D Gillmore; Andor W J M Glaudemans; Mazen A Hanna; Bouke P C Hazenberg; Arnt V Kristen; Raymond Y Kwong; Mathew S Maurer; Giampaolo Merlini; Edward J Miller; James C Moon; Venkatesh L Murthy; C Cristina Quarta; Claudio Rapezzi; Frederick L Ruberg; Sanjiv J Shah; Riemer H J A Slart; Hein J Verberne; Jamieson M Bourque Journal: J Nucl Cardiol Date: 2019-12 Impact factor: 5.952
Authors: Keyur B Shah; Anit K Mankad; Adam Castano; Olakunle O Akinboboye; Phillip B Duncan; Icilma V Fergus; Mathew S Maurer Journal: Circ Heart Fail Date: 2016-06 Impact factor: 8.790
Authors: David L Narotsky; Adam Castano; Jonathan W Weinsaft; Sabahat Bokhari; Mathew S Maurer Journal: Can J Cardiol Date: 2016-05-13 Impact factor: 5.223
Authors: Song Soo Kim; Sung Min Ko; Sang Il Choi; Bo Hwa Choi; Arthur E Stillman Journal: Int J Cardiovasc Imaging Date: 2016-05-02 Impact factor: 2.357