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SHR Neuro Krebs Kardio Lipid Stoffw Microb

Debonnaire, P; L'Hoyes, W; Donal, E; Verheyen, N; Vervloet, D; Dujardin, K; Pouleur, AC; Dulgheru, R; Sarli, Issa, V; Droogmans, S; Jurcut, R; Regeer, M; Dupont, M; Bondue, A; Timmermans, P; Bohyn, A; Christiaen, E; Wyseure, N; Bezard, M; Zach, D; Schwegel, N; Knapen, R; Buytaert, L; de, Marneffe, N; Adam, R; Ajmone, Marsan, N; Tavernier, R; Buysschaert, I; Trenson, S.
Global Longitudinal Strain for Prognostic Staging in Wild-Type Transthyretin Cardiac Amyloidosis.
Circ Cardiovasc Imaging. 2025; e018862 Doi: 10.1161/CIRCIMAGING.125.018862 [OPEN ACCESS]
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Co-Autor*innen der Med Uni Graz

Schwegel Nora

Verheyen Nicolas Dominik

Zach David

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Abstract:

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 ATTRwt-CM patient cohort, 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.

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