Global Longitudinal Strain for Prognostic Staging in Wild-Type Transthyretin Cardiac Amyloidosis.
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Debonnaire Philippe, L'Hoyes Wouter, Donal Erwan, Verheyen Nicolas, Vervloet Delphine, Dujardin Karl, Pouleur Anne-Catherine, Dulgheru Raluca, Sarli Issa Victor, Droogmans Steven, Jurcut Ruxandra, Regeer Madelien, Dupont Matthias, Bondue Antoine, Timmermans Philippe, Bohyn Alexandre, Christiaen Emma, Wyseure Nicolas, Bezard Mélanie, Zach David, Schwegel Nora, Knapen Robbe, Buytaert Lars, de Marneffe Nils, Adam Robert, Ajmone Marsan Nina, Tavernier René, Buysschaert Ian, Trenson Sander
Аннотация
METHODS: A multicentric, international ATTRwt-CM patient cohort with baseline GLS (distribution quartiles), evaluated by echocardiography, was studied, related to all-cause mortality.
RESULTS: The study comprised 816 patients with ATTRwt-CM, median age of 81.5 years, 83% males, and 72% tafamidis initiated. During a 2.2-year 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<0.001). 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 (P<0.001). The median GLS -12.8% cutoff predicted 1-year mortality with 74% sensitivity, 52% specificity (area under the curve, 0.73 [95% CI, 0.66-0.80]; P<0.001). GLS was the only independent echocardiographic and strong mortality predictor, independent of other predictors, including age, New York Heart Association class symptoms, NAC stage, and tafamidis treatment (hazard ratio, 1.08 [95% CI, 1.04-1.12]; P<0.001), also when restricted to 591 tafamidis-treated subjects (hazard ratio, 1.15 [95% CI, 1.08-1.22]; P<0.001). Baseline GLS -12.8% cutoff value provided further prognostic discriminative ability for mortality within each NAC disease stage stratum (all P<0.050). Likelihood ratio test indicated incremental prognostic value of GLS (staging) over baseline NAC staging (P<0.001).
CONCLUSIONS: GLS is a strong, independent mortality predictor in ATTRwt-CM, irrespective of tafamidis treatment, that may be an adjunct or complementary to biomarker staging.