Baseline GLS -12.8% predicted mortality in ATTRwt-CM with 73% sensitivity and 60% specificity, demonstrating strong independent prognostic value over NAC staging.
Does global longitudinal strain (GLS) staging predict all-cause mortality in patients with wild-type transthyretin amyloid cardiomyopathy independently of NAC biomarker staging?
Global longitudinal strain is a strong, independent predictor of mortality in wild-type transthyretin amyloid cardiomyopathy and provides incremental prognostic value over standard biomarker staging.
Absolute Event Rate: 0% vs 0%
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%, p0.001). Median survival per baseline GLS quartile stage 1(-15.8%), 2(-15.8 to -12.9%), 3(-12.8 to -10.0%) and 4(GLS -10.0%) was not met, 6.7, 4.6 and 3.4 years, respectively (p0.001). GLS -12.8% cut-off predicted mortality with 73% sensitivity, 60% specificity, AUC 0.70 (95%CI 0.66-0.74, p0.001). GLS was the only echocardiographic and a strong mortality predictor, independent of other predictors, including age, NYHA class symptoms, NAC disease stage and tafamidis treatment (HR 1.07, 95%CI 1.03-1.11, p=0.001), also when restricted to 591 tafamidis treated subjects (HR 1.14, 95%CI 1.07-1.21, p0.001). Baseline GLS -12.8% cut-off value provided further prognostic discriminative ability for mortality within each NAC disease stage stratum (all p0.050). Likelihood ratio test indicated incremental prognostic value of GLS (staging) over baseline NAC staging (p0.001). Conclusions GLS is a strong, independent mortality predictor in ATTRwt-CM, including under tafamidis treatment, that may be used as an adjunct or alternative to biomarker staging. GLS stages survival
Debonnaire et al. (Thu,) reported a other. Baseline GLS -12.8% predicted mortality in ATTRwt-CM with 73% sensitivity and 60% specificity, demonstrating strong independent prognostic value over NAC staging.