eprintid: 27018 rev_number: 12 eprint_status: archive userid: 1589 dir: disk0/00/02/70/18 datestamp: 2019-08-28 08:43:36 lastmod: 2019-10-01 13:40:57 status_changed: 2019-08-28 08:43:36 type: article metadata_visibility: show creators_name: Arenja, Nisha creators_name: Andre, Florian creators_name: Riffel, Johannes H. creators_name: aus dem Siepen, Fabian creators_name: Hegenbart, Ute creators_name: Schönland, Stefan creators_name: Kristen, Arnt V. creators_name: Katus, Hugo A. creators_name: Buss, Sebastian J. title: Prognostic value of novel imaging parameters derived from standard cardiovascular magnetic resonance in high risk patients with systemic light chain amyloidosis subjects: ddc-610 divisions: i-910100 keywords: Immunoglobulin light chain amyloidosis, Cardiovascular magnetic resonance, Long axis strain, Myocardial contraction fraction, Longitudinal function, Prognosis abstract: 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. date: 2019 publisher: BioMed Central id_scheme: DOI ppn_swb: 1677934085 own_urn: urn:nbn:de:bsz:16-heidok-270180 language: eng bibsort: ARENJANISHPROGNOSTIC2019 full_text_status: public publication: Journal of Cardiovascular Magnetic Resonance volume: 21 number: 53 place_of_pub: London pagerange: 1-12 issn: 1532-429X citation: 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 document_url: https://archiv.ub.uni-heidelberg.de/volltextserver/27018/1/12968_2019_Article_564.pdf