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Open Access Highly Accessed Research article

Prognostic implication of late gadolinium enhancement on cardiac MRI in light chain (AL) amyloidosis on long term follow up

Raymond Q Migrino12*, Richard Christenson2, Aniko Szabo3, Megan Bright1, Seth Truran1 and Parameswaran Hari4

Author Affiliations

1 Cardiovascular Medicine Division, Medical College of Wisconsin, 9200 W Wisconsin Avenue, Milwaukee WI 53226, USA

2 Radiology Department, Medical College of Wisconsin, 9200 W Wisconsin Avenue, Milwaukee WI 53226, USA

3 Biostatistics Division, Medical College of Wisconsin, 9200 W Wisconsin Avenue, Milwaukee WI 53226, USA

4 Hematology-Oncology Division, Medical College of Wisconsin, 9200 W Wisconsin Avenue, Milwaukee WI 53226, USA

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BMC Medical Physics 2009, 9:5  doi:10.1186/1756-6649-9-5

Published: 5 May 2009

Abstract

Background

Light chain amyloidosis (AL) is a rare plasma cell dyscrasia associated with poor survival especially in the setting of heart failure. Late gadolinium enhancement (LGE) on cardiac MRI was recently found to correlate with myocardial amyloid deposition but the prognostic role is not established. The aim is to determine the prognostic significance of LGE in AL by comparing long term survival of AL patients with and without LGE.

Methods

Twenty nine consecutive patients (14 females; 62 ± 11 years) with biopsy-proven AL undergoing cardiac MRI with gadolinium as part of AL workup were included. Survival was prospectively followed 29 months (median) following MRI and compared between those with and without LGE by Kaplan-Meier and log-rank analyses.

Results

LGE was positive in 23 subjects (79%) and negative in 6 (21%). Left ventricular ejection fraction was 66 ± 17% in LGE-positive and 69 ± 12% in LGE-negative patients (p = 0.8). Overall 1-year mortality was 36%. On follow-up, 14/23 LGE-positive and none of LGE-negative patients died (log rank p = 0.0061). Presenting New York Heart Association heart failure class was also associated with poor survival (p = 0.0059). Survival between two LGE groups stratified by heart failure class still showed a significant difference by a stratified log-rank test (p = 0.04).

Conclusion

Late gadolinium enhancement is common and is associated with poor long-term survival in light chain amyloidosis, even after adjustment for heart failure class presentation. The prognostic significance of late gadolinium enhancement in this disease may be useful in patient risk-stratification.