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Published on: April 8, 2013
Impact of regional left ventricular function on outcome for patients with AL amyloidosis
Dan Liu1, Kai Hu, Markus Niemann
1Department of Internal Medicine I, University of Würzburg, Würzburg, Germany.
Insights
Reduced left ventricular (LV) deformation in AL amyloidosis patients with LV hypertrophy indicates a worse prognosis. Mid-septum longitudinal strain is a key predictor of survival in these patients.
Area of Science:
- Cardiology
- Medical Imaging
- Cardiac Physiology
Background:
- Cardiac involvement in light-chain (AL) amyloidosis significantly impacts patient outcomes.
- Advanced non-invasive imaging techniques can aid in predicting prognosis by assessing regional cardiac function.
Purpose of the Study:
- To investigate left ventricular (LV) deformation changes in patients with AL amyloidosis and LV hypertrophy.
- To determine the impact of LV deformation on patient outcomes.
Main Methods:
- Speckle tracking imaging (STI) was used to assess LV longitudinal, circumferential, and radial peak systolic strains (Ssys) in 44 AL amyloidosis patients and 30 controls.
- Patients were categorized into compensated and decompensated groups and followed for survival.
Main Results:
- Left ventricular ejection fraction (EF) was preserved, but longitudinal Ssys (LSsys) was reduced in both patient groups.
- Decompensated patients had significantly reduced survival (35% vs. 78% in compensated).
- Reduced LSsys, particularly in mid-septum segments, correlated with increased mortality risk, with LSsys <11% indicating a 4.8-fold higher risk.
Conclusions:
- Reduced mid-septum deformation is a significant indicator of poor prognosis in AL amyloidosis patients with LV hypertrophy.
- Mid-septum LSsys and NYHA class are independent predictors of survival.
Objectives:
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.
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