Prognostic Utility of Regional Longitudinal Strain in Patients With Immunoglobulin Light-Chain Cardiac Amyloidosis: A Report From the J-CASE Multicenter Survey.

BACKGROUND: This study aimed to evaluate the prognostic impact of left ventricular (LV) regional longitudinal strain (LS) in patients with immunoglobulin light-chain (AL) cardiac amyloidosis. METHODS AND RESULTS: The study population was part of the Japan Cardiac Amyloidosis Survey of typical Echocardiographic findings (J-CASE) study - a multicenter, retrospective study with 18 participating institutions in Japan. From a total of 324 patients with histologically proven cardiac amyloidosis enrolled between 2000 and 2020, we enrolled 81 patients with AL cardiac amyloidosis in the present study. During a median follow up of 278 days (interquartile range 63-1,133 days), 43 all-cause deaths occurred. LV-apical LS was significantly and independently associated with all-cause death after adjusting for conventional echocardiographic findings (hazard ratio 0.86; 95% confidence interval; P<0.05). Receiver operating characteristic curve analysis showed that the area under the curve for LV-apical LS for all-cause death was 68% and the best cut-off value for LV-apical LS was 15.9% (sensitivity 71%; specificity 54%). Kaplan-Meier analysis demonstrated a significantly higher risk of all-cause death in patients with low LV-apical LS (<15.9%, n=46) than in those with high LV-apical LS (≥15.9%, n=35; log-rank test P<0.05). CONCLUSIONS: Low LV-apical LS is a significant and independent prognostic factor in patients with AL cardiac amyloidosis.