Prevalence and Outcomes of Concomitant Aortic Stenosis and Cardiac Amyloidosis.

BACKGROUND Older patients with severe aortic stenosis (AS) are increasingly identified to have cardiac amyloidosis (CA). It is unknown whether dual AS-CA has worse outcomes or results in futility of transcatheter aortic valve replacement (TAVR). OBJECTIVE To identify clinical characteristics and outcomes of AS-CA compared to lone AS. METHODS TAVR referrals at three international sites underwent blinded research-corelab 99mTc-DPD bone scintigraphy (Perugini Grade-0 negative, 1-3 increasingly positive) prior to intervention. Transthyretin-CA (ATTR) was diagnosed by DPD and absence of a clonal immunoglobulin, and light-chain-CA (AL) via tissue biopsy. National registries captured all-cause mortality. RESULTS 407 patients (83.4±6.5 years, 49.8% male) were recruited. DPD was positive in n=48 (11.8%, Grade-1 3.9%[n=16] Grade-2/3 7.9%[n=32]); AL was diagnosed in one Grade-1. Grade-2/3 patients had worse functional capacity, biomarkers (NT-proBNP/hsTnT), and bi-ventricular remodeling. A clinical score (RAISE) using left-ventricular Remodeling (hypertrophy/diastolic dysfunction), Age, Injury (hsTnT), Systemic involvement, and Electrical abnormalities (RBBB/low-voltages) was developed to predict AS-CA presence (AUC 0.86, 95%CI 0.78-0.94, p<0.001). Heart Team decision (DPD-blinded) resulted in TAVR (333[81.6%]), surgical-AVR (10[2.5%]), or medical management (65[15.9%]). After median 1.7 years, 23% of patients had died. 1-year mortality was worse in all-comers AS-CA (Grade-1-3) than lone AS (24.5 vs 13.9%, p=0.05). TAVR improved survival versus medical management with AS-CA survival post-TAVR no different to lone AS (p=0.36). CONCLUSION Dual pathology of AS-CA is common in older AS patients and can be predicted clinically. AS-CA has worse clinical presentation and a trend towards worse prognosis, unless treated. TAVR should therefore not be withheld in AS-CA.