Transcatheter Aortic Valve Replacement is Appropriate for Patients with Aortic Stenosis and Cardiac Amyloidosis

Older patients with severe aortic stenosis are being diagnosed more frequently with transthyretin cardiac amyloidosis (ATTR-CA). Further research is needed to determine if patients who have both aortic stenosis and ATTR-CA experience worse outcomes or receive clinical benefit from transcatheter aortic valve replacement (TAVR).

Christian Nitsche, MD, of the Medical University of Vienna, Austria, and his colleagues attempted to identify diagnostic predictors and outcomes of concomitant aortic stenosis and ATTR-CA compared with aortic stenosis alone.

At 3 international sites, severe aortic stenosis patients who had received referrals for TAVR underwent laboratory and clinical assessment, including transthoracic echocardiography with strain analysis, ECG, 6-minute walk test, and blinded research technetium-99m 3,3-diphosphono-1,2-propanodicarboxylic acid (DPD) bone scintigraphy (Perugini grade-0 negative, 1-3 increasingly positive) prior to valve intervention. Bone scintigraphy and unremarkable serum/urine free light chain assessment were used to diagnose ATTR-CA. Endomyocardial biopsy was used to diagnose light chain cardiac amyloidosis. National registries were used to obtain all-cause mortality information.

In total, the study recruited 408 patients, of whom 49.8% were male, with a mean age of 83.4 ± 6.5 years. In 11.6% of patients, DPD bone scintigraphy was positive (N = 47, grade 1 in 15 patients [3.7%] and grade 2/3 in 32 patients [7.9%]). A positive bone scintigraphy was associated with a history of carpal tunnel syndrome (odds ratio [OR], 1.6; 95% confidence interval [CI], 1.1-2.3; P = .02), voltage/mass ratio (OR, 0.4; 95% CI, 0.2-0.9; P = .02), and QRS duration (OR, 2.5; 95% CI, 1.1-5.5; P = .02). Biopsy-proven concomitant aortic stenosis and ATTR-CA was confirmed in an additional 2 cases. Based on the treating clinicians’ decisions (they were blinded to the bone scintigraphy results), 65 (15.9%) patients were treated via medical management, 333 (81.6%) patients received TAVR, and 10 (2.5%) patients received surgical aortic valve replacement.

After a median of 1.7 years of follow-up, 22.9% of patients died. Patients with concomitant aortic stenosis and ATTR-CA had a significantly worse 1-year mortality outcome than those with aortic stenosis alone (24.5% vs 13.9%; P = .045).

Compared with patients who received medical management, improved mortality outcomes were observed in both lone aortic stenosis and concomitant aortic stenosis and ATTR-CA groups, although there was no significant difference between these 2 cohorts following valve intervention (P = .7).

Concomitant ATTR-CA is common in older patients who have aortic stenosis. Outcomes for patients with aortic stenosis and ATTR-CA are slightly worse than for patients who have aortic stenosis alone, but not if treated via valve intervention. The results of this study suggest that TAVR should be used in patients with aortic stenosis and ATTR-CA.


Nitsche C, Scully P, Patel K, et al. Dal pathology of severe aortic stenosis and cardiac amyloidosis: multi-center study of prevalence and outcome. Presented at: American Heart Association Scientific Sessions 2020; November 13-20, 2020. Abstract MP246-2020.

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