TY - JOUR
T1 - Utility of doppler myocardial imaging, cardiac biomarkers, and clonal immunoglobulin genes to assess left ventricular performance and stratify risk following peripheral blood stem cell transplantation in patients with systemic light chain amyloidosis (AL)
AU - Bellavia, Diego
AU - Abraham, Roshini S.
AU - Pellikka, Patricia A.
AU - Dispenzieri, Angela
AU - Burnett, John C.
AU - Al-Zahrani, Ghormallah B.
AU - Green, Tammy D.
AU - Manske, Michelle K.
AU - Gertz, Morie A.
AU - Miller, Fletcher A.
AU - Abraham, Theodore P.
N1 - Funding Information:
Drs. Bellavia and Abraham contributed equally to this report. This study was supported in part by grant R21 HL 76513 from the National Institutes of Health (Bethesda, MD) and ASE Research Award 2008-2009 from the American Society of Echocardiography (Morrisville, NC).
PY - 2011/4
Y1 - 2011/4
N2 - Background: Cardiac dysfunction is a well-recognized complication of light chain amyloidosis (AL). Autologous stem cell transplant (auto-SCT) has emerged as a successful treatment modality for AL patients. In this study, we examined the effect of clonal immunoglobulin light chain genes (VL), which encodes the immunoglobulin light chain protein that ultimately forms amyloid, on cardiac function, in the context of auto-SCT and its impact on overall survival. Methods: Longitudinal Doppler myocardial imaging parameters along with cardiac biomarkers were used to assess for cardiac function pre and post auto-SCT. Results: VL gene analysis revealed that Vl genes, in particular VlVI, were associated with worse cardiac function parameters than Vk genes. Clonal VL genes appeared to have an impact on left ventricular (LV) function post-transplant and also influenced mortality, with specific VL gene families associated with lower survival. Another key predictor of mortality in this report was change in tricuspid regurgitant flow velocity following auto-SCT. Correlations were also observed between systolic strain rate, systolic strain and VL genes associated with amyloid formation. Conclusions: Clonal VL gene usage influences global cardiac function in AL, with patients having VlVI and VlII-III-associated amyloid more severely affected than those having Vk or VlI amyloid. Pulsed wave tissue Doppler imaging along with immunoglobulin gene analysis offers novel insights into prediction of mortality and cardiac dysfunction in AL after auto-SCT.
AB - Background: Cardiac dysfunction is a well-recognized complication of light chain amyloidosis (AL). Autologous stem cell transplant (auto-SCT) has emerged as a successful treatment modality for AL patients. In this study, we examined the effect of clonal immunoglobulin light chain genes (VL), which encodes the immunoglobulin light chain protein that ultimately forms amyloid, on cardiac function, in the context of auto-SCT and its impact on overall survival. Methods: Longitudinal Doppler myocardial imaging parameters along with cardiac biomarkers were used to assess for cardiac function pre and post auto-SCT. Results: VL gene analysis revealed that Vl genes, in particular VlVI, were associated with worse cardiac function parameters than Vk genes. Clonal VL genes appeared to have an impact on left ventricular (LV) function post-transplant and also influenced mortality, with specific VL gene families associated with lower survival. Another key predictor of mortality in this report was change in tricuspid regurgitant flow velocity following auto-SCT. Correlations were also observed between systolic strain rate, systolic strain and VL genes associated with amyloid formation. Conclusions: Clonal VL gene usage influences global cardiac function in AL, with patients having VlVI and VlII-III-associated amyloid more severely affected than those having Vk or VlI amyloid. Pulsed wave tissue Doppler imaging along with immunoglobulin gene analysis offers novel insights into prediction of mortality and cardiac dysfunction in AL after auto-SCT.
KW - AL amyloidosis
KW - Cardiac biomarkers
KW - Doppler myocardial imaging
KW - Immunoglobulin genes
KW - LV function
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U2 - 10.1016/j.echo.2011.01.003
DO - 10.1016/j.echo.2011.01.003
M3 - Article
C2 - 21315556
AN - SCOPUS:79953163546
SN - 0894-7317
VL - 24
SP - 444-454.e2
JO - Journal of the American Society of Echocardiography
JF - Journal of the American Society of Echocardiography
IS - 4
ER -