Prevalence and Outcomes of Concomitant Aortic Stenosis and Cardiac Amyloidosis.

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 = 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/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 = 0.05). TAVR improved survival versus medical management; AS-CA survival post-TAVR did not differ from lone 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.

Is stress-only imaging practical when a 1-day stress-rest 99mTc-tetrofosmin protocol is used?

OBJECTIVES: To investigate whether a significant number of normal 1-day stress-rest 99mTc-tetrofosmin myocardial perfusion scintigraphy (MPS) studies can be identified from the low-dose stress acquisition alone, and whether technical staff can find such studies reliably. METHODS: The supervising consultant and four technologists independently graded the stress acquisitions from 200 consecutive MPS studies using a five-point scale. Studies were classified as normal or abnormal according to the final clinical report based on the completed stress-rest protocol. RESULTS: Between 31 and 62 studies (16-31%) were classified as definitely normal from the stress acquisition alone, of which 0-4 (0-9%) proved abnormal on the final report. Of stress studies graded definitely normal by each technologist, the consultant disagreed in 13-34% of cases. Of 78 stress studies graded definitely normal by at least one technologist, 6% turned out to be abnormal and the consultant disagreed in 33%. When there was agreement between at least two technologists (57 studies), the rates fell to 4% and 21% respectively. CONCLUSIONS: Technologists could make the decision to avoid a resting study in up to 30% of patients following a normal low-dose stress acquisition. The supervising nuclear cardiologist would disagree in perhaps one in five cases, even when there was consensus between two or more technologists. These patients would suffer minor inconvenience by being recalled for a rest acquisition on a second day, but there would be important savings in time and radiation exposure for the majority.