Nitsche, C;
Scully, PR;
Patel, KP;
Kammerlander, A;
Koschutnik, M;
Dona, C;
Wollenweber, T;
... Treibel, TA; + view all
(2021)
Prevalence and Outcomes of Concomitant Aortic Stenosis and Cardiac Amyloidosis.
Journal of the American College of Cardiology
, 77
(2)
pp. 128-139.
10.1016/j.jacc.2020.11.006.
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Abstract
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.
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