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

Abstract

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 (ρspearman = 0.35; p < 0.001) and deltaGWI (ρspearman = -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.

Systemic Vascular Resistance and Myocardial Work Analysis in Hypertrophic Cardiomyopathy and Transthyretin Cardiac Amyloidosis with Preserved Left Ventricular Ejection Fraction

“…This further confirms their cardiovascular system impairment is differently influenced by interstitial amyloid fibril accumulation than by genetic disorders featuring primary HCM [ 15 , 16 , 21 , 24 , 33 , 34 ]. Conversely, preserved MW indices and weakly higher (or normal) SVR c , as in two ATTR patients in our Class D, may suggest a less compromised systemic condition or early stage of disease, more likely to obtain benefit from specific therapy [ 23 , 24 , 28 , 32 , 35 ].…”

“…Even though all ATTR patients in this study were classified as HFpEF, the present results reinforce the pathophysiological theory that amyloidosis is a systemic illness in which the vascular compartment progressively deteriorates alongside the heart, somehow consistent with their clinical status. On the other hand, most ATTR patients present with autonomic dysfunction and BP variability, with a challenging vascular reactivity that affects their quality of life and prognosis [ 16 , 32 , 33 ]. This further confirms their cardiovascular system impairment is differently influenced by interstitial amyloid fibril accumulation than by genetic disorders featuring primary HCM [ 15 , 16 , 21 , 24 , 33 , 34 ].…”