CMR Imaging With Rapid Visual T1 Assessment Predicts Mortality in Patients Suspected of Cardiac Amyloidosis

doi:10.1016/j.jcmg.2013.09.019

JACC: Cardiovascular Imaging

Volume 7, Issue 2, February 2014, Pages 143-156

https://doi.org/10.1016/j.jcmg.2013.09.019 Get rights and content

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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.

Key Words

amyloid

magnetic resonance imaging

delayed enhancement

Abbreviations and Acronyms

cardiac amyloidosis

CAD

coronary artery disease

CMR

cardiac magnetic resonance

DE-CMR

delayed-enhancement cardiac magnetic resonance

ECG

electrocardiogram

EMB

endomyocardial biopsy

hyperenhancement

IQR

interquartile range

left ventricular

LVH

left ventricular hypertrophy

Cited by (0)

: Funding for the research was provided in part by National Institutes of Health grant RO1-HL64726 (Dr. R. Kim). Dr. White is a clinician scientist with the Heart and Stroke Foundation of Ontario, Canada. Dr. Patel has received research grants from Johnson and Johnson, AstraZeneca, the National Heart, Lung and Blood Institute, and the Agency for Healthcare Research and Quality; and has served on the advisory board for Genzyme and Jansen. Drs. Judd and R. Kim are inventors of a U.S. patent on Delayed Enhancement CMR, which is owned by Northwestern University. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.