Prognostic significance of semiautomatic quantification of left ventricular long axis shortening in systemic light-chain amyloidosis

Amyloid. 2015 Mar;22(1):45-53. doi: 10.3109/13506129.2014.992515. Epub 2014 Dec 10.


Aims: To assess left ventricular long axis shortening (LAS) in patients with AL amyloidosis as a potential predictor for outcome.

Methods and 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%.

Conclusion: 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.

Keywords: Amyloidosis; echocardiography; long axis shortening; longitudinal strain; prognosis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Amyloidosis / diagnostic imaging*
  • Amyloidosis / mortality
  • Amyloidosis / pathology
  • Female
  • Heart Ventricles / diagnostic imaging
  • Heart Ventricles / pathology*
  • Humans
  • Kaplan-Meier Estimate
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Organ Size
  • Prognosis
  • Proportional Hazards Models
  • ROC Curve
  • Retrospective Studies
  • Ultrasonography