Canadian experience with the RAISE score to identify patients at high risk for cardiac amyloidosis in a TAVI population

ATTR cardiac amyloidosis (CA) has been increasingly found in patients with severe aortic stenosis undergoing transcatheter aortic valve implantation (TAVI). In order to better identify patients at risk, the RAISE score (based on Remodeling, Age, Injury, Systemic and Electrical disorders) has been recently published but not tested in a real care setting. To assess: 1) the distribution of the patients according to their RAISE score in our TAVI population; and 2) if this score could be fine-tuned to improve its screening performance. A multidisciplinary committee (MDC) reviewed the medical chart of every patient (≤ 90 years) who underwent a TAVI within the past 3 years at our institution and decided whether or not a patient should be screened for CA. The decision was based not only on the presence of red flags for CA but also on the potential direct clinical benefit for the patient. The original RAISE score (oRAISE) (Fig. 1) was then slightly modified (mRAISE) (Fig. 1) to improve its usability or applicability, then retrospectively tested in the same cohort using a score ≥ 3 as a threshold for screening (best global predictive value in the original publication). Finally, based on our local experience, we devised and tested an alternative version of the score, enriched by additional criteria (eRAISE) b, to refine patient selection for screening. The MDC reviewed 251 TAVI patients files (mean age 79 ± 7 y, 55% male), 49 were invited for screening (19.5%), 6 refused. mRAISE score (mS) was 0 in 86 (34.3%) patients, 1 in 69 (27.5%), 2 in 44 (17.5%), 3 in 29 (11.5%), 4 in 13 (5.2%) and ≥ 5 in 10 (4%). Among patients with mS ≥ 3 (n = 52, 20.7%), 27 (51.9%) got screened based on the MDC's judgment only, with 2 positive cases (grade 2 and 3 PYP scintigraphies). Among mS < 3 patients (n = 199, 79.3%), 16 (8%) got screened by the MDC with no grade 2 or 3 PYP scintigraphy. The eRAISE score (eS) reclassified 30 patients with mS ≥ 3 as “not to be screened” and 7 mS < 3 as “to be screened” resulting in only 29 (11.5%) patients classified as high risk for CA. Among those, 14 (48.3%) were screened, among which the two grades 2 and 3 cases. The prospective use of mRAISE would have triaged one fifth of our cohort as high risk for CA, including all positive cases. eRAISE would have triaged as high risk 44% less patients than mRAISE with similar sensitivity. However, the eRAISE score requires validation in an independent cohort, especially considering the low prevalence of CA in our TAVI cohort.