title: Prognostic value of novel imaging parameters derived from standard cardiovascular magnetic resonance in high risk patients with systemic light chain amyloidosis
creator: Arenja, Nisha
creator: Andre, Florian
creator: Riffel, Johannes H.
creator: aus dem Siepen, Fabian
creator: Hegenbart, Ute
creator: Schönland, Stefan
creator: Kristen, Arnt V.
creator: Katus, Hugo A.
creator: Buss, Sebastian J.
subject: ddc-610
subject: 610 Medical sciences Medicine
description: Background: The differentiated assessment of functional parameters besides morphological changes is essential for the evaluation of prognosis in systemic immunoglobulin light chain (AL) amyloidosis.    Methods: Seventy-four subjects with AL amyloidosis and presence of late gadolinium enhancement (LGE) pattern typical for cardiac amyloidosis were analyzed. Long axis strain (LAS) and myocardial contraction fraction (MCF), as well as morphological and functional markers, were measured. The primary endpoint was death, while death and heart transplantation served as a composite secondary endpoint.    Results: After a median follow-up of 41 months, 29 out of 74 patients died and 10 received a heart transplant. Left ventricular (LV) functional parameters were reduced in patients, who met the composite endpoint (LV ejection fraction 51% vs. 61%, LAS − 6.9% vs − 10%, GLS − 12% vs − 15% and MCF 42% vs. 69%; p <  0.001 for all). In unadjusted univariate analysis, LAS (HR = 1.05, p <  0.001) and MCF (HR = 0.96, p <  0.001) were associated with reduced transplant-free survival. Kaplan-Meier analyses showed a significantly lower event-free survival in patients with reduced MCF. MCF and LAS performed best to identify high risk patients for secondary endpoint (Log-rank test p <  0.001) in a combined model. Using sequential Cox regression analysis, the addition of LAS and MCF to LV ejection fraction led to a significant increase in the predictive power of the model (χ2 (df = 1) = 28.2, p <  0.001).    Conclusions: LAS and MCF as routinely available and robust CMR-derived parameters predict outcome in LGE positive AL amyloidosis. Patients with impaired LV function in combination with reduced LAS and MCF are at the highest risk for death and heart transplantation.
publisher: BioMed Central
date: 2019
type: Article
type: info:eu-repo/semantics/article
type: NonPeerReviewed
format: application/pdf
identifier: https://archiv.ub.uni-heidelberg.de/volltextserverhttps://archiv.ub.uni-heidelberg.de/volltextserver/27018/1/12968_2019_Article_564.pdf
identifier: DOI:
identifier: urn:nbn:de:bsz:16-heidok-270180
identifier:   Arenja, Nisha ; Andre, Florian ; Riffel, Johannes H. ; aus dem Siepen, Fabian ; Hegenbart, Ute ; Schönland, Stefan ; Kristen, Arnt V. ; Katus, Hugo A. ; Buss, Sebastian J.  (2019) Prognostic value of novel imaging parameters derived from standard cardiovascular magnetic resonance in high risk patients with systemic light chain amyloidosis.  Journal of Cardiovascular Magnetic Resonance, 21 (53).  pp. 1-12.  ISSN 1532-429X     
relation: https://archiv.ub.uni-heidelberg.de/volltextserver/27018/
rights: info:eu-repo/semantics/openAccess
rights: Please see front page of the work (Sorry, Dublin Core plugin does not recognise license id)
language: eng