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)

作者：Bellavia Diego; Abraham Roshini S^*; Pellikka Patricia A; Dispenzieri Angela; Burnett John C Jr; Al Zahrani Ghormallah B; Green Tammy D; Manske Michelle K; Gertz Morie A; Miller Fletcher A Jr; Abraham Theodore P

来源：Journal of the American Society of Echocardiography, 2011, 24(4): 444-U118.

DOI：10.1016/j.echo.2011.01.003

• Dopplermyocardial imaging
• ALamyloidosis
• Immunoglobulin genes
• LVfunction
• Cardiac biomarkers

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 VIVI and VIII-III-associated amyloid more severely affected than those having Vk or VII 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. (J Am Soc Echocardiogr 2011;24:444-54.)