Impact of regional left ventricular function on outcome for patients with AL amyloidosis

Liu, Dan; Hu, Kai; Niemann, Markus; Herrmann, Sebastian; Cikes, Maja; Stork, Stefan; Beer, Meinrad; Gaudron, Daniel; Morbach, Caroline; Knop, Stefan; Geissinger, Eva; Ertl, George; Bijnens, Bart; Weidemann, Frank

doi:http://dx.doi.org/10.1371/journal.pone.0056923

Impact of regional left ventricular function on outcome for patients with AL amyloidosis

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Liu D, Hu K, Niemann M, Herrmann S, Cikes M, Stork S, Beer M, Daniel Gaudron P, Morbach C, Knop S, Geissinger E, Ertl G, Bijnens B, Weidemann F. Impact of regional left ventricular function on outcome for patients with AL amyloidosis. PLoS ONE. 2013;8(3):1-12. DOI: 10.1371/journal.pone.0056923

Abstract

Objectives: The aim of this study was to explore the left ventricular (LV) deformation changes and the potential impact of/ndeformation on outcome in patients with proven light-chain (AL) amyloidosis and LV hypertrophy./nBackground: Cardiac involvement in AL amyloidosis patients is associated with poor outcome. Detecting regional cardiac/nfunction by advanced non-invasive techniques might be favorable for predicting outcome./nMethods: LV longitudinal, circumferential and radial peak systolic strains (Ssys) were assessed by speckle tracking imaging/n(STI) in 44 biopsy-proven systemic AL amyloidosis patients with LV hypertrophy (CA) and in 30 normal controls. Patients/nwere divided into compensated (n = 18) and decompensated (n = 26) group based on clinical assessment and followed-up/nfor a median period of 345 days./nResults: Ejection fraction (EF) was preserved while longitudinal Ssys (LSsys) was significantly reduced in both compensated/nand decompensated groups. Survival was significantly reduced in decompensated group (35% vs. compensated 78%,/nP = 0.001). LSsys were similar in apical segments and significantly reduced in basal segments between two patient groups./nLSsys at mid-segments were significantly reduced in all LV walls of decompensated group. Patients were further divided into/n4 subgroups according to the presence or absence of reduced LSsys in no (normal), only basal (mild), basal and mid/n(intermediate) and all segments of the septum (severe). This staging revealed continuously worse prognosis in proportion to/nincreasing number of segments with reduced LSsys (mortality: normal 14%, mild 27%, intermediate 67%, and severe 64%)./nMid-septum LSsys,11% suggested a 4.8-fold mortality risk than mid-septum LSsys$11%. Multivariate regression analysis/nshowed NYHA class and mid-septum LSsys were independent predictors for survival./nConclusions: Reduced deformation at mid-septum is associated with worse prognosis in systemic amyloidosis patients with/nLV hypertrophy.