Prevalence and Outcomes of Concomitant Aortic Stenosis and Cardiac Amyloidosis

JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

卷:77

Article

Nitsche, Christian¹  Scully, Paul R.^2,3  Patel, Kush P.^2,4  Kammerlander, Andreas A.¹  Koschutnik, Matthias¹  Dona, Carolina¹  Wollenweber, Tim⁵  Ahmed, Nida^2,4  Thornton, George D.^2,4  Kelion, Andrew D.⁶  Sabharwal, Nikant⁶  Newton, James D.⁶  Ozkor, Muhiddin⁴  Kennon, Simon⁴  Mullen, Michael⁴  Lloyd, Guy^2,4,7  Fontana, Marianna⁸  Hawkins, Philip N.⁸  Pugliese, Francesca^2,7  Menezes, Leon J.^4,9  Moon, James C.^2,4  Mascherbauer, Julia¹  Treibel, Thomas A.^2,4 

[1] Med Univ Vienna, Dept Internal Med 2, Div Cardiol, Vienna, Austria

[2] UCL, Inst Cardiovasc Sci, London, England

[3] Guys & St Thomas NHS Fdn Trust, Cardiol Dept, London, England

[4] St Bartholomews Hosp, Barts Heart Ctr, London EC1A 7BE, England

[5] Med Univ Vienna, Dept Nucl Med, Vienna, Austria

[6] John Radcliffe Hosp, Oxford, England

[7] Queen Mary Univ London, London, England

[8] Natl Amyloid Ctr, London, England

[9] UCL ULCH NIHR Biomed Res Ctr, London, England

关键词: aortic stenosis;    cardiac amyloidosis;    TAVR;   

DOI  :  10.1016/j.jacc.2020.11.006

来源: Elsevier

【 摘 要 】

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 tone AS. METHODS Patients who were referred for TAVR at 3 international sites underwent blinded research core laboratory (99m)technetium-3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) bone scintigraphy (Perugini grade O: 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 = 16]; grade 2/3: 7.9% [n = 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/tow 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 alt patients with AS-CA (grade:1 to 3) than those with tone AS (24.5% vs. 13.9%; p = 0.05). TAVR improved survival versus medical management; AS-CA survival post-TAVR did not differ from tone AS (p = 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. (C) 2021 The Authors. Published by Elsevier on behalf of the American College of Cardiology Foundation.

【 授权许可】

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