Affiliation:
1. Cardiology, ASST Spedali Civili di Brescia, Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health University of Brescia Brescia Italy
2. Health Sciences Interdisciplinary Center, Scuola Superiore Sant'Anna Pisa Italy
3. Cardiology Division, Fondazione Toscana Gabriele Monasterio Pisa Italy
Abstract
AbstractAimsSeveral scores were developed to help the diagnosis of cardiac amyloidosis (CA). The most recent one, being the Mayo transthyretin amyloidosis cardiomyopathy (ATTR‐CM) score, was not externally validated. We compared the diagnostic performance of the ATTR‐CM score with previous tools (increased wall thickness [IWT] score, AMYLoidosis Index [AMYLI] score, and cardiac biomarkers) in a cohort of patients evaluated for a suspicion of CA.Methods and resultsWe analysed 362 consecutive patients referred to a third‐level centre for suspected CA. Overall, 132 (36%) had transthyretin CA (ATTR‐CA), and 91 (25%) immunoglobulin light chain CA (AL‐CA); CA was excluded in 139 (38%). ATTR‐CM score had a good diagnostic performance to distinguish ATTR‐CA from AL‐CA or no CA, with an area under the curve (AUC) of 0.795 (95% confidence interval [CI] 0.747–0.842, p < 0.001), and ATTR‐CA from no CA (AUC 0.822, 95% CI 0.774–0.871, p < 0.001). Results were consistent in both patients with preserved (AUC 0.787, 95% CI 0.726–0.848, p < 0.001), and reduced or mildly reduced ejection fraction (AUC 0.790, 95% CI 0.709–0.871, p < 0.001). The ATTR‐CM score showed a better discrimination compared to IWT and AMYLI score to distinguish ATTR‐CA from AL‐CA or no CA (p = 0.002), but not to distinguish ATTR‐CA from no CA (p = 0.270). Diagnostic accuracy was significantly higher for the ATTR‐CM score as compared to the rule‐in cut‐off of high‐sensitivity troponin T.ConclusionThe Mayo ATTR‐CM score has a good performance in identifying patients with ATTR‐CA, with also better discrimination power when compared to other scores and biomarkers.