Key result
The eRAISE score achieves ~0.95 AUC for detecting ATTR-CA in severe aortic stenosis.
Why the study?
The coexistence of transthyretin cardiac amyloidosis and severe aortic stenosis presents a diagnostic challenge, prompting evaluation of its prevalence and diagnostic model accuracy.
Do diagnostic risk scores accurately detect transthyretin cardiac amyloidosis in patients with severe aortic stenosis?
Cohort (n=104)
No
Do diagnostic risk scores accurately detect transthyretin cardiac amyloidosis in patients with severe aortic stenosis?
Answers are grounded in this paper and cite their sources.
The eRAISE risk score demonstrates high diagnostic accuracy (AUC=0.948) for detecting concomitant ATTR-CA, which is present in 18% of patients with severe aortic stenosis.
Introduction The coexistence of transthyretin cardiac amyloidosis (ATTR-CA) and severe aortic stenosis (AS) presents a diagnostic challenge. This study aimed to determine the prevalence of this dual pathology and to assess the effectiveness of diagnostic models in enhancing detection accuracy. Methods We conducted a prospective study of 104 consecutive patients with severe AS (aortic valve area <1 cm 2 ). Comprehensive evaluation included clinical and laboratory assessments, electrocardiography, transthoracic echocardiography, planar whole-body bone scintigraphy, and chest single-photon emission computed tomography/computed tomography using technetium-99m-labelled 3,3-diphosphono-1,2-propanodicarboxylic acid tracer. Patients were grouped according to the presence of ATTR-CA, and the diagnostic utility of the RAISE score, its variations, and the T-AMYLO score was evaluated. Results Nineteen (18%) patients with AS also had ATTR-CA (ATTR-CA-AS). They were significantly older (82.6 ± 7.4 vs. 76 ± 6.9, p < 0.001), more often had arrhythmic and conduction disorders (atrial fibrillation, implanted pacemakers), and had a reduced left ventricular ejection fraction (44.6% vs. 54.7%, p = 0.03). Patients with ATTR-CA-AS more frequently presented with the low-gradient/low-flow phenotype of AS (73.7%, p = 0.009). Both NT-proBNP and troponin levels were significantly higher in patients with ATTR-CA-AS (4174.0 vs. 1,238 pg/mL; p < 0.001; and 59.6 vs. 23.3 ng/L, p < 0.001, respectively). After a 6-month follow-up, a similar number of deaths occurred in both groups, and no surgical aortic valve replacement was performed in the ATTR-CA-AS population. The eRAISE model demonstrated the highest diagnostic accuracy (AUC=0.948). Conclusions ATTR-CA is commonly observed in patients with severe AS. Implementing risk scores in routine practice could enhance the diagnostic accuracy of ATTR-CA in patients with AS, given their strong diagnostic performance and ease of assessment.
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Katarzyna et al. (2026) conducted a cohort in Severe aortic stenosis (n=104). eRAISE diagnostic score vs. Other diagnostic scores (oRAISE, mRAISE, T-AMYLO) was evaluated on Diagnostic accuracy (AUC) for identifying ATTR-CA (AUC 0.948, 95% CI 0.901-0.995). The eRAISE diagnostic model demonstrated the highest accuracy for identifying transthyretin cardiac amyloidosis among patients with severe aortic stenosis, achieving an AUC of 0.948.
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