Coexisting aortic stenosis-cardiac amyloidosis tied to poorer survival in older patients

Concomitant pathology of aortic stenosis (AS) and cardiac amyloidosis (CA) occurs frequently in older AS patients and has worse clinical presentation and prognosis unless treated, suggests a study. Hence, transcatheter aortic valve replacement (TAVR) must not be denied in AS-CA patients.

Four hundred seven patients (mean age, 83.4 years; 49.8 percent men) referred for TAVR at three 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) by tissue biopsy. National registries were used to assess all-cause mortality.

Forty-eight patients (11.8 percent) had positive DPD (grade 1: 3.9 percent; grade 2/3: 7.9 percent). One patient was diagnosed with AL grade 1. Those with grade 2/3 had worse functional capacity, biomarkers (N-terminal probrain natriuretic peptide and high-sensitivity troponin T), and biventricular remodeling.

The presence of AS-CA (area under the curve, 0.86, 95 percent confidence interval, 0.78–0.94; p<0.001) was predicted using a clinical score (RAISE) that utilized left ventricular remodeling, age, injury, systemic involvement, and electrical abnormalities. Decisions by the heart team (DPD-blinded) resulted in TAVR (81.6 percent), surgical AVR (2.5 percent), or medical management (15.9 percent).

Twenty-three percent of patients died after a median of 1.7 years. Patients with AS-CA (grade 1–3) had worse 1-year mortality than those with lone AS (24.5 percent vs 13.9 percent; p=0.05). Furthermore, TAVR prolonged survival compared with medical management. AS-CA survival post-TAVR was not different from lone AS (p=0.36).