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  • Oral presentation
  • Open Access

Delayed hyper-enhancement cardiac magnetic resonance provides incremental prognostic value in patients with cardiac amyloidosis

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Journal of Cardiovascular Magnetic Resonance200911 (Suppl 1) :O65

https://doi.org/10.1186/1532-429X-11-S1-O65

  • Published:

Keywords

  • Cardiac Magnetic Resonance
  • Left Atrial
  • High Diagnostic Accuracy
  • Cardiac Amyloidosis
  • Myocardial Performance Index

Introduction

Patients with cardiac amyloidosis (CA) have an unfavorable, albeit a variable prognosis. In patients with documented cardiac amyloidosis (CA), delayed hyper-enhancement-cardiac magnetic resonance (DHE-CMR) has been demonstrated to have a high diagnostic accuracy. However, its prognostic utility in CA has not been determined.

Purpose

We sought to determine the incremental prognostic value of DHE-CMR in CA.

Methods

We studied 47 consecutive patients with suspected CA (mean age 63 ± 13 years, 70% men, 55% with NYHA class > 2) that underwent electrocardiography (ECG), transthoracic echocardiography (TTE), DHE-CMR (Siemens 1.5 T scanner, Erlangen, Germany) and biopsy (38 endomyocardial, 9 extracardiac) between 1/05 and 7/08. Low voltage on ECG was defined as sum of S wave in lead V1 + R wave in lead V5 or V6 < 15 mm. Measured TTE parameters included left atrial size, interventricular septal thickness, speckled appearance, E/A ratio, E/E' ratio, stage of diastology, deceleration time (msec) and myocardial performance index [(isovolumic contraction time + isovolumic relaxation time)/ejection time]. DHE-CMR images were obtained in standard long and short axis orientations (covering the entire LV), after injection of Gadolinium dimenglumine using an inversion recovery spoiled gradient echo sequence: TE 4 msec, TR 8 msec, flip angle 30°, bandwidth 140 Hz/pixel, 23 k-space lines acquired every other RR-interval, field of view (varied from 228–330 in the x-direction and 260–330 in the y-direction) and matrix size (varied from 140–180 in the x-direction and 256 in the y-direction). CMR was considered positive in the presence of DHE of entire subendocardium with extension into the neighboring myocardium. All-cause mortality was ascertained.

Results

At baseline, 59% patients had low voltage on ECG, while 67% had deceleration time < 150 msec and 53% had E/E' > 15 (both on Doppler echocardiography). Mean MPI, left ventricular ejection fraction and interventricular septal thickness were 0.51 ± 0.3, 51% ± 13 and 1.5 cm ± 0.5, respectively. At up to 1-year after biopsy, there were 9 (19%) deaths. Results of Cox Proportional Hazard survival analysis are shown in Table 1. On univariate Kaplan-Meier survival analysis, presence of DHE on CMR was associated with worse 1-year survival (log rank statistic p-value = 0.03, Figure 1).
Table 1

Cox proportional hazard analysis of various clinical and noninvasive imaging predictors of long-term mortality in patients with biopsy proven cardiac amyloidosis

 

Univariate Analysis

 

Multivariate Analysis

Variable

χ2

p value

p value

Age

3.8

0.05

0.10

Gender

0.15

0.69

 

New York Heart Association Class

3.3

0.07

0.16

Low voltage on electrocardiogram

0.67

0.41

 

Left atrial size > 20 cm2

0.76

0.39

 

Left ventricular ejection fraction

2.21

0.14

 

Interventricular septal thickness

1.7

0.19

 

E/E' > 15 on Doppler echocardiography

1.12

0.29

 

Deceleration time on Doppler echocardiography ≤ 150 msec

1.41

0.23

 

Myocardial performance index

2.10

0.15

 

Diastology grade

0.35

0.55

 

Delayed hyperenhancement on cardiac magnetic resonance

4.91

0.03

0.02

Chi-square for multivariate model = 12.27, p-value = 0.007

Figure 1

Conclusion

Presence of DHE on CMR is associated with worse 1-year survival in CA. Along with a high diagnostic accuracy; DHE-CMR adds incremental prognostic value in CA, independent of other variables.

Authors’ Affiliations

(1)
Cleveland Clinic, Cleveland, OH, USA

Copyright

© Austin et al; licensee BioMed Central Ltd. 2009

This article is published under license to BioMed Central Ltd.

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