title: Prognostic significance of semiautomatic quantification of left ventricular long axis shortening in systemic light-chain amyloidosis.
abstract: OBJECTIVE
  To assess left ventricular long axis shortening (LAS) in patients with AL amyloidosis as a potential predictor for outcome.
  
  
  RESULTS
  We performed a de novo echocardiographic analysis of LAS in 120 patients with biopsy-proven AL amyloidosis evaluated at first presentation before specific treatment. Additionally, 47 control subjects were analyzed retrospectivly. LAS was measured using a semiautomatic tissue motion annular displacement software algorithm (TMAD). LAS was significantly better than ejection fraction (EF) (p < 0.0001) and M-mode-derived mitral annular plane systolic excursion (MAPSE) (p < 0.05) discriminating AL patients from control subjects, while being non-inferior compared to tissue Doppler-derived peak systolic mitral annular velocity. One year outcome analysis in patients with AL amyloidosis showed that LAS remained the only significant echocardiographic parameter (HR:0.76; p < 0.005) in a multivariable Cox regression model of echocardiographic values. In a comprehensive clinical model, LAS (HR:0.72, p < 0.0001), cardiac troponin-T (HR:2.86, p < 0.01) and free light chain difference (HR:1.00; p < 0.05) were independently associated with the outcome. Assessment of LAS led to a significant integrated discrimination improvement and offered incremental information compared to EF and biomarkers. The cut-off value for LAS discriminating the endpoint was 5.8%.
  
  
  CONCLUSIONS
  LAS was an independent predictor of survival within the first year and offers incremental information in patients with AL amyloidosis evaluated prior to specific treatment.
authors.affiliation: Department of Cardiology .
authors.forename: Johannes H
authors.initials: JH
authors.lastname: Riffel
authors.forename: Derliz
authors.initials: D
authors.lastname: Mereles
authors.forename: Mostafa
authors.initials: M
authors.lastname: Emami
authors.forename: Grigorios
authors.initials: G
authors.lastname: Korosoglou
authors.forename: Arnt V
authors.initials: AV
authors.lastname: Kristen
authors.forename: Matthias
authors.initials: M
authors.lastname: Aurich
authors.forename: Andreas
authors.initials: A
authors.lastname: Voss
authors.forename: Stefan O
authors.initials: SO
authors.lastname: Schonland
authors.forename: Ute
authors.initials: U
authors.lastname: Hegenbart
authors.forename: Stefan E
authors.initials: SE
authors.lastname: Hardt
authors.forename: Hugo A
authors.initials: HA
authors.lastname: Katus
authors.forename: Sebastian J
authors.initials: SJ
authors.lastname: Buss
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000000981:diagnostic imaging* / Q000401:mortality / Q000473:pathology; D005260:Female; D006352:Heart Ventricles / Q000000981:diagnostic imaging / Q000473:pathology*; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009929:Organ Size; D011379:Prognosis; D016016:Proportional Hazards Models; D012372:ROC Curve; D012189:Retrospective Studies; D014463:Ultrasonography
keywords: Amyloidosis; echocardiography; long axis shortening; longitudinal strain; prognosis