Cardiac amyloidosis Echo-Score for patients with severe aortic stenosis. The AMY-TAVI trial

Abstract Background and Purpose A longitudinal strain pattern characterized by apical sparing (RELAPS>1) has been identified as indicative of cardiac amyloidosis (CA). We conducted an analysis to assess its diagnostic accuracy in individuals with severe aortic stenosis (AS) and explored novel echocardiographic variables for predictive value. Methods All patients diagnosed with AS who underwent transcatheter aortic valve implantation (TAVI) were prospectively enrolled. 2D-Speckle-tracking echocardiography was utilized to assess myocardial deformation parameters. Subsequent to TAVI, screening for CA was conducted through 99mTc-DPD scintigraphy and protein electrophoresis. Results A total of 324 patients were included (mean age 81.5 ± 5.8 years, 51% women). Among them, 38 individuals (11.7%) exhibited cardiac uptake in scintigraphy: 14 patients (4.3%) with grade 1, 13(4%) grade 2, and 11(3.4%) grade 3. RELAPS>1 was more prevalent in patients with AS-CA (74%vs.44%,p=0.006). We propose an echocardiographic prediction model (GRAM score) for CA in the context of AS that is more sensitive and specific than RELAPS >1 alone. This model incorporates the left ventricular mass index, maximum transvalvular aortic pressure gradient, RELAPS>1, and age (AUC: 0.85, 95% CI: 0.77–0.93). Conclusions While RELAPS >1 is more commonly observed in individuals with AS-CA, it is noteworthy that this pattern can also manifest in nearly half of AS patients without CA, diminishing its utility as a standalone screening tool. In light of this, our study puts forth a more sensitive and specific prediction score for identifying CA in patients with severe AS.GRAM score for predicting AS-CA