Echocardiographic Markers of Cardiac Response to Therapy in Patients with Light Chain Amyloidosis

The aim of this study was to identify echocardiographic parameters which correlate with cardiac response to treatment in patients with light chain cardiac amyloidosis (AL-CA).

We identified 39 patients with AL-CA treated at City of Hope and divided them into two cohorts; cardiac responders as defined by improvement in global longitudinal strain (GLS) over time and cardiac non-responders as defined by no change or worsening in GLS. We then compared baseline demographics and echocardiographic parameters between the two groups.

There were 16 cardiac responders and 23 cardiac non-responders identified. Correlation between changes in GLS was compared with change in other echocardiographic parameters. The change in GLS correlated with changes in left ventricular ejection fraction (LVEF) (r = -0.44, p < 0.01), stroke volume index (r = -0.05, p < 0.01), posterior wall thickness (r = 0.41, p = 0.01), right ventricular (RV) free wall strain (r = 0.66, p < 0.01), right ventricular four-chamber longitudinal strain (r = 0.67, p < 0.01), left atrial conduit strain (r = 0.46, p < 0.01), left atrial reservoir strain (r = -0.63, p < 0.01), and left atrial contractile strain (r = 0.48, p < 0.01). At less than 1 year, there was a positive correlation between change in GLS and change in RV free wall strain (r = 0.73, p < 0.01) and change in right ventricular four-chamber longitudinal strain (r = 0.75, p < 0.01).

Improvement in GLS correlated with improvement in RV and left atrial strain measurements in AL-CA patients receiving treatment. Changes in RV strain were seen early, within 1 year of treatment. Our study suggests that strain imaging in conjunction with cardiac biomarkers can provide a comprehensive assessment of cardiac response.