title: Impact of regional left ventricular function on outcome for patients with AL amyloidosis.
abstract: OBJECTIVE
  The 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.
  
  
  BACKGROUND
  Cardiac 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.
  
  
  METHODS
  LV 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.
  
  
  RESULTS
  Ejection 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 LSsys<11% suggested a 4.8-fold mortality risk than mid-septum LSsys≥11%. Multivariate regression analysis showed NYHA class and mid-septum LSsys were independent predictors for survival.
  
  
  CONCLUSIONS
  Reduced deformation at mid-septum is associated with worse prognosis in systemic amyloidosis patients with LV hypertrophy.
authors.affiliation: Department of Internal Medicine I, University of Würzburg, Würzburg, Germany.
authors.forename: Dan
authors.initials: D
authors.lastname: Liu
authors.forename: Kai
authors.initials: K
authors.lastname: Hu
authors.forename: Markus
authors.initials: M
authors.lastname: Niemann
authors.forename: Sebastian
authors.initials: S
authors.lastname: Herrmann
authors.forename: Maja
authors.initials: M
authors.lastname: Cikes
authors.forename: Stefan
authors.initials: S
authors.lastname: Störk
authors.forename: Meinrad
authors.initials: M
authors.lastname: Beer
authors.forename: Philipp Daniel
authors.initials: PD
authors.lastname: Gaudron
authors.forename: Caroline
authors.initials: C
authors.lastname: Morbach
authors.forename: Stefan
authors.initials: S
authors.lastname: Knop
authors.forename: Eva
authors.initials: E
authors.lastname: Geissinger
authors.forename: Georg
authors.initials: G
authors.lastname: Ertl
authors.forename: Bart
authors.initials: B
authors.lastname: Bijnens
authors.forename: Frank
authors.initials: F
authors.lastname: Weidemann
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000150:complications / Q000401:mortality* / Q000503:physiopathology* / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000150:complications / Q000401:mortality* / Q000503:physiopathology* / Q000601:surgery; D008297:Male; D008875:Middle Aged; D013318:Stroke Volume; D015996:Survival Rate; D016277:Ventricular Function, Left