Left ventricular myocardial work improves in response to treatment and is associated with survival among patients with light chain cardiac amyloidosis

Aims: Complete hematologic response to treatment for light chain cardiac amyloidosis (AL-CA) may lead to improvement of myocardial function and better outcomes. We sought to evaluate the effect of response to treatment for AL-CA on echocardiographic indices of myocardial deformation and work, and their prognostic significance.Methods and results: Sixty-one patients treated for AL were enrolled and underwent echocardiographic assessment at baseline and at 1-year. Patients were stratified according to hematologic response as complete or not complete responders. A significant reduction in median NT-proBNP (2,771 to 1,486 pg/mL; p < 0.001), posterior wall thickness (PWT) (13 to 12 mm; p = 0.002), and an increase in global work index (GWI) (1,115 to 1,356 mmHg%; p = 0.018) was observed at 1-year. Patients with complete response (CR) had a more pronounced decrease in intraventricular septum thickness (14.2 to 12.0 mm; p = 0.006), improved global longitudinal strain (GLS) (-11.6 to -13.1%; p for interaction = 0.045), increased global constructive work (1,245 to 1,436 mmHg%; p = 0.008) and global work index (926 to 1250 mmHg%, p = 0.002) compared to non-CR. Furthermore, deltaGLS (rho spearman = 0.35; p < 0.001) and deltaGWI (rho spearman = -0.32; p = 0.02) correlated with delta NT-proBNP. Importantly, patients with GLS and GWI response had a better prognosis (log-rank p = 0.048 and log-rank p = 0.007, respectively). After adjustment for Mayo stage, gender, and response status, deltaGLS (HR = 1.404, p = 0.046 per 1% increase) and deltaGWI (HR = 0.996, p = 0.042 per 1mmHg% increase) were independent predictors of survival.Conclusions: Complete hematologic response to treatment is associated with improved left ventricular myocardial work indices and their change is associated with improved survival in AL-CA.{GRAPHIACAL ABSTRACT}