PLoS ONE (Jan 2013)

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

  • Dan Liu,
  • Kai Hu,
  • Markus Niemann,
  • Sebastian Herrmann,
  • Maja Cikes,
  • Stefan Störk,
  • Meinrad Beer,
  • Philipp Daniel Gaudron,
  • Caroline Morbach,
  • Stefan Knop,
  • Eva Geissinger,
  • Georg Ertl,
  • Bart Bijnens,
  • Frank Weidemann

DOI
https://doi.org/10.1371/journal.pone.0056923
Journal volume & issue
Vol. 8, no. 3
p. e56923

Abstract

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ObjectivesThe aim of this study was to explore the left ventricular (LV) deformation changes and the potential impact of deformation on outcome in patients with proven light-chain (AL) amyloidosis and LV hypertrophy.BackgroundCardiac involvement in AL amyloidosis patients is associated with poor outcome. Detecting regional cardiac function by advanced non-invasive techniques might be favorable for predicting outcome.MethodsLV longitudinal, circumferential and radial peak systolic strains (Ssys) were assessed by speckle tracking imaging (STI) in 44 biopsy-proven systemic AL amyloidosis patients with LV hypertrophy (CA) and in 30 normal controls. Patients were divided into compensated (n = 18) and decompensated (n = 26) group based on clinical assessment and followed-up for a median period of 345 days.ResultsEjection fraction (EF) was preserved while longitudinal Ssys (LSsys) was significantly reduced in both compensated and decompensated groups. Survival was significantly reduced in decompensated group (35% vs. compensated 78%, P = 0.001). LSsys were similar in apical segments and significantly reduced in basal segments between two patient groups. LSsys at mid-segments were significantly reduced in all LV walls of decompensated group. Patients were further divided into 4 subgroups according to the presence or absence of reduced LSsys in no (normal), only basal (mild), basal and mid (intermediate) and all segments of the septum (severe). This staging revealed continuously worse prognosis in proportion to increasing number of segments with reduced LSsys (mortality: normal 14%, mild 27%, intermediate 67%, and severe 64%). Mid-septum LSsysConclusionsReduced deformation at mid-septum is associated with worse prognosis in systemic amyloidosis patients with LV hypertrophy.