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

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
figure1

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.

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Correspondence to Bethany A Austin.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (https://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Austin, B.A., Flamm, S., Rodriguez, E.R. et al. Delayed hyper-enhancement cardiac magnetic resonance provides incremental prognostic value in patients with cardiac amyloidosis. J Cardiovasc Magn Reson 11, O65 (2009). https://doi.org/10.1186/1532-429X-11-S1-O65

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Keywords

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