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

<h4>Objectives</h4>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.<h4>Background</h4>Cardiac involvement in AL amyloi...

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Autores principales: 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
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Publicado: Public Library of Science (PLoS) 2013
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spelling oai:doaj.org-article:383aaf2963ed48998f200e7d8c39eabd2021-11-18T07:54:14ZImpact of regional left ventricular function on outcome for patients with AL amyloidosis.1932-620310.1371/journal.pone.0056923https://doaj.org/article/383aaf2963ed48998f200e7d8c39eabd2013-01-01T00:00:00Zhttps://www.ncbi.nlm.nih.gov/pmc/articles/pmid/23520459/?tool=EBIhttps://doaj.org/toc/1932-6203<h4>Objectives</h4>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.<h4>Background</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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.<h4>Conclusions</h4>Reduced deformation at mid-septum is associated with worse prognosis in systemic amyloidosis patients with LV hypertrophy.Dan LiuKai HuMarkus NiemannSebastian HerrmannMaja CikesStefan StörkMeinrad BeerPhilipp Daniel GaudronCaroline MorbachStefan KnopEva GeissingerGeorg ErtlBart BijnensFrank WeidemannPublic Library of Science (PLoS)articleMedicineRScienceQENPLoS ONE, Vol 8, Iss 3, p e56923 (2013)
institution DOAJ
collection DOAJ
language EN
topic Medicine
R
Science
Q
spellingShingle Medicine
R
Science
Q
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
Impact of regional left ventricular function on outcome for patients with AL amyloidosis.
description <h4>Objectives</h4>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.<h4>Background</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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.<h4>Conclusions</h4>Reduced deformation at mid-septum is associated with worse prognosis in systemic amyloidosis patients with LV hypertrophy.
format article
author 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
author_facet 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
author_sort Dan Liu
title Impact of regional left ventricular function on outcome for patients with AL amyloidosis.
title_short Impact of regional left ventricular function on outcome for patients with AL amyloidosis.
title_full Impact of regional left ventricular function on outcome for patients with AL amyloidosis.
title_fullStr Impact of regional left ventricular function on outcome for patients with AL amyloidosis.
title_full_unstemmed Impact of regional left ventricular function on outcome for patients with AL amyloidosis.
title_sort impact of regional left ventricular function on outcome for patients with al amyloidosis.
publisher Public Library of Science (PLoS)
publishDate 2013
url https://doaj.org/article/383aaf2963ed48998f200e7d8c39eabd
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