Prevalence and Outcomes of Concomitant Aortic Stenosis and Cardiac Amyloidosis
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Volume
77
Pagination
128 - 139
DOI
10.1016/j.jacc.2020.11.006
Journal
JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY
Issue
ISSN
0735-1097
Metadata
Show full item recordAbstract
BACKGROUND: Older patients with severe aortic stenosis (AS) are increasingly identified as having cardiac amyloidosis
(CA). It is unknown whether concomitant AS-CA has worse outcomes or results in futility of transcatheter aortic valve
replacement (TAVR).
OBJECTIVES: This study identified clinical characteristics and outcomes of AS-CA compared with lone AS.
METHODS: Patients who were referred for TAVR at 3 international sites underwent blinded research core laboratory
99mtechnetium-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) bone scintigraphy (Perugini grade 0: negative; grades 1
to 3: increasingly positive) before intervention. Transthyretin-CA (ATTR) was diagnosed by DPD and absence of a clonal
immunoglobulin, and light-chain CA (AL) was diagnosed via tissue biopsy. National registries captured all-cause mortality.
RESULTS: A total of 407 patients (age 83.4 6.5 years; 49.8% men) were recruited. DPD was positive in 48 patients
(11.8%; grade 1: 3.9% [n ¼ 16]; grade 2/3: 7.9% [n ¼ 32]). AL was diagnosed in 1 patient with grade 1. Patients with grade
2/3 had worse functional capacity, biomarkers (N-terminal pro-brain natriuretic peptide and/or high-sensitivity troponin
T), and biventricular remodeling. A clinical score (RAISE) that used left ventricular remodeling (hypertrophy/diastolic
dysfunction), age, injury (high-sensitivity troponin T), systemic involvement, and electrical abnormalities (right bundle
branch block/low voltages) was developed to predict the presence of AS-CA (area under the curve: 0.86; 95% confidence
interval: 0.78 to 0.94; p < 0.001). Decisions by the heart team (DPD-blinded) resulted in TAVR (333 [81.6%]), surgical
AVR (10 [2.5%]), or medical management (65 [15.9%]). After a median of 1.7 years, 23% of patients died. One-year
mortality was worse in all patients with AS-CA (grade: 1 to 3) than those with lone AS (24.5% vs. 13.9%; p ¼ 0.05). TAVR
improved survival versus medical management; AS-CA survival post-TAVR did not differ from lone AS (p ¼ 0.36).
CONCLUSIONS: Concomitant pathology of AS-CA is common in older patients with AS and can be predicted clinically.
AS-CA has worse clinical presentation and a trend toward worse prognosis, unless treated. Therefore, TAVR should not
be withheld in AS-CA. (J Am Coll Cardiol 2021;77:128–39) © 2021 The Authors. Published by Elsevier on behalf of
the American College of Cardiology Foundation. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
Authors
Nitsche, C; Scully, PR; Patel, KP; Kammerlander, AA; Koschutnik, M; Dona, C; Wollenweber, T; Ahmed, N; Thornton, GD; Kelion, ADCollections
- NIHR Advanced Imaging [386]