Global longitudinal strain for prognostic staging in wild-type transthyretin cardiac amyloidosis, including under tafamidis treatment

Abstract Background A formal prognostic staging system in wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM) based on echocardiographic imaging is currently lacking. Purpose We evaluated the prognostic performance of GLS staging in a large ATTRwt-CM patient cohort, relative to National Amyloidosis Centre (NAC) biomarker staging, including under tafamidis treatment. Methods A multicentric, international ATTRwt-CM patient cohort with baseline GLS evaluated by echocardiography was studied, related to all-cause mortality. Results The study comprised 816 ATTRwt-CM patients, median age 81.5 years, 83% males, 72% tafamidis initiated. During 2.2 years median follow-up, 29.7% of patients died. GLS worsened with increasing NAC disease stage (I: -14.3%, II: -11.6%, III: -11.4%, p