Multi-imaging characterisation of cardiac phenotype in different types of amyloidosis

Abstract Background Bone scintigraphy is extremely valuable when assessing patients with suspected cardiac amyloidosis (CA), but the clinical significance and associated phenotype of different degrees myocardial tracer uptake across types yet to be defined. Purpose We sought define phenotypes varying on bone scintigraphy, multiple systemic using extensive characterisation comprising biomarkers, echocardiographic magnetic resonance (CMR) imaging. Methods A total 296 (117 immunoglobulin light-chain [AL] amyloidosis, 165 transthyretin [ATTR] 7 apolipoprotein-A1-amyloidosis [AApoAI],and apolipoprotein-A4-amyloidosis [AApoA4]) underwent deep their phenotype. Results AL-amyloidosis grade 0 radiotracer spanned spectrum CMR findings from no evidence CA characteristic features CA, while 1–3 always produced features. In ATTR-amyloidosis burden strongly correlated (correlation between derived extracellular volume: R=0.88, 95% CI [0.84–0.91], P<0.001), except in Ser77Tyr variant. AApoAI-amyloidosis presented 0–1 uptake, unique disproportionate right sided involvement such as ventricular (RV) atrial RV free wall thickening, tricuspid valve thickening dysfunction. Within our cohort, AApoAIV-amyloidosis CMR. All 1 had (n=48, 100%), only variant (n=5, 11.4%). Following exclusion AApoAI, a showing or an volume >0.40 patient sensitivity specificity 100% for diagnosing AL-amyloidosis. Conclusion Deep range grades has identified clear differences each type. The distinctive characteristics cohort allowed development diagnostic pathway help differentials individual patient, following comprehensive assessment Funding Acknowledgement Type funding sources: None.