Prognostic and added value of two-dimensional global longitudinal strain for prediction of survival in patients with light chain amyloidosis undergoing autologous hematopoietic cell transplantation Journal Article


Authors: Pun, S. C.; Landau, H. J.; Riedel, E. R.; Jordan, J.; Yu, A. F.; Hassoun, H.; Chen, C. L.; Steingart, R. M.; Liu, J. E.
Article Title: Prognostic and added value of two-dimensional global longitudinal strain for prediction of survival in patients with light chain amyloidosis undergoing autologous hematopoietic cell transplantation
Abstract: Background Autologous hematopoietic cell transplantation (HCT) is a first-line therapy for prolonging survival in patients with light-chain (AL) amyloidosis. Cardiac involvement is the most important determinant of survival. However, patients with advanced cardiac involvement have often been excluded from HCT because of high risk for transplantation-related mortality and poor overall survival. Whether baseline left ventricular global longitudinal strain (GLS) can provide additional risk stratification and predict survival after HCT in this high-risk population remains unclear. The aim of this study was to evaluate the prognostic implication of baseline GLS and the added value of GLS beyond circulating cardiac biomarkers for risk stratification in patients with AL amyloidosis undergoing HCT. Methods Eighty-two patients with newly diagnosed AL amyloidosis who underwent upfront HCT between January 2007 and April 2014 were included in the study. Clinical, echocardiographic, and serum cardiac biomarker data were collected at baseline and 12 months following HCT. GLS measurements were performed using a vendor-independent offline system. The median follow-up time for survivors was 58 months. Results Sixty-four percent of patients were in biomarker-based Mayo stage II or III. GLS, brain natriuretic peptide, troponin, and mitral E/A ratio were identified as the strongest predictors of survival (P <.0001). Other predictors included sex, creatinine, free AL, wall thickness, and ejection fraction. Mayo stage was significantly associated with outcome, with 5-year survival of 93%, 72% and 31% in stage I, II, and III patients, respectively. GLS of 17% was identified as the value that best discriminated survivors from nonsurvivors, and the application of this cutoff value provided further mortality risk stratification within each Mayo stage. Conclusions GLS is a strong predictor of survival in patients with AL amyloidosis undergoing HCT, potentially providing incremental value over serum cardiac biomarkers for risk stratification. GLS should be considered as a standard parameter along with serum cardiac biomarkers when evaluating eligibility for HCT or other investigational therapies. © 2017 American Society of Echocardiography
Keywords: survival; adult; aged; survival rate; major clinical study; disease marker; follow up; biological marker; protein blood level; image analysis; observer variation; risk factor; risk assessment; image quality; cardiovascular risk; sex difference; age distribution; echocardiography; image processing; heart left ventricle ejection fraction; brain natriuretic peptide; autologous hematopoietic stem cell transplantation; heart function; autologous hematopoietic cell transplantation; cardiovascular parameters; troponin; al amyloidosis; arterial wall thickness; human; male; female; article; prognostic assessment; global longitudinal strain; serum cardiac biomarkers; heart left ventricular global longitudinal strain
Journal Title: Journal of the American Society of Echocardiography
Volume: 31
Issue: 1
ISSN: 0894-7317
Publisher: Mosby Elsevier  
Date Published: 2018-01-01
Start Page: 64
End Page: 70
Language: English
DOI: 10.1016/j.echo.2017.08.017
PROVIDER: scopus
PUBMED: 29111123
PMCID: PMC5985664
DOI/URL:
Notes: Article -- Export Date: 6 February 2018 -- Source: Scopus
Altmetric Score
MSK Authors
  1. Jennifer Liu
    39 Liu
  2. Richard M Steingart
    116 Steingart
  3. Carol Chen
    26 Chen
  4. Hani Hassoun
    149 Hassoun
  5. Heather Jolie Landau
    189 Landau
  6. Anthony Yu
    37 Yu
  7. Shawn Clinton Pun
    9 Pun
  8. Jonathan Nichol Jordan
    2 Jordan