TY - JOUR
T1 - Global Longitudinal Strain for Prognostic Staging in Wild-Type Transthyretin Cardiac Amyloidosis.
AU - Debonnaire, P
AU - L'Hoyes, W
AU - Donal, E
AU - Verheyen, Nicolas Dominik
AU - Vervloet, D
AU - Dujardin, K
AU - Pouleur, AC
AU - Dulgheru, R
AU - Sarli Issa, V
AU - Droogmans, S
AU - Jurcut, R
AU - Regeer, M
AU - Dupont, M
AU - Bondue, A
AU - Timmermans, P
AU - Bohyn, A
AU - Christiaen, E
AU - Wyseure, N
AU - Bezard, M
AU - Zach, David
AU - Schwegel, Nora
AU - Knapen, R
AU - Buytaert, L
AU - de Marneffe, N
AU - Adam, R
AU - Ajmone Marsan, N
AU - Tavernier, R
AU - Buysschaert, I
AU - Trenson, S
PY - 2026/1
Y1 - 2026/1
N2 - BACKGROUND: A formal prognostic staging system in wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM), based on echocardiographic imaging, is lacking. We evaluated the prognostic performance of global longitudinal strain (GLS) staging in a large cohort of patients with ATTRwt-CM, including under tafamidis treatment and relative to National Amyloidosis Center (NAC) biomarker staging.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.
AB - BACKGROUND: A formal prognostic staging system in wild-type transthyretin amyloid cardiomyopathy (ATTRwt-CM), based on echocardiographic imaging, is lacking. We evaluated the prognostic performance of global longitudinal strain (GLS) staging in a large cohort of patients with ATTRwt-CM, including under tafamidis treatment and relative to National Amyloidosis Center (NAC) biomarker staging.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.
UR - https://www.webofscience.com/wos/woscc/full-record/WOS:001667308100004
UR - https://pubmed.ncbi.nlm.nih.gov/41342126
UR - https://pmc.ncbi.nlm.nih.gov/articles/PMC12825696
U2 - 10.1161/CIRCIMAGING.125.018862
DO - 10.1161/CIRCIMAGING.125.018862
M3 - Research article
C2 - 41342126
SN - 1941-9651
VL - 19
SP - e018862
JO - Circulation: Cardiovascular Imaging
JF - Circulation: Cardiovascular Imaging
IS - 1
M1 - e018862
ER -