Abstract
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.
| Original language | English |
|---|---|
| Pages (from-to) | 444-454 |
| Number of pages | 11 |
| Journal | Journal of the American Society of Echocardiography |
| Volume | 24 |
| Issue number | 4 |
| DOIs | |
| Publication status | Published - Apr 2011 |
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Keywords
- AL amyloidosis
- Cardiac biomarkers
- Doppler myocardial imaging
- Immunoglobulin genes
- LV function
ASJC Scopus subject areas
- Radiology Nuclear Medicine and imaging
- Cardiology and Cardiovascular Medicine
Cite this
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). / Bellavia, Diego; Abraham, Roshini S.; Pellikka, Patricia A.; Dispenzieri, Angela; Burnett, John C.; Al-Zahrani, Ghormallah B.; Green, Tammy D.; Manske, Michelle K.; Gertz, Morie A.; Miller, Fletcher A.; Abraham, Theodore P.
In: Journal of the American Society of Echocardiography, Vol. 24, No. 4, 04.2011, p. 444-454.Research output: Contribution to journal › Article
}
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.
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
UR - http://www.scopus.com/inward/record.url?scp=79953163546&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=79953163546&partnerID=8YFLogxK
U2 - 10.1016/j.echo.2011.01.003
DO - 10.1016/j.echo.2011.01.003
M3 - Article
C2 - 21315556
AN - SCOPUS:79953163546
VL - 24
SP - 444
EP - 454
JO - Journal of the American Society of Echocardiography
JF - Journal of the American Society of Echocardiography
SN - 0894-7317
IS - 4
ER -