Identifying Cardiac Amyloid in Aortic Stenosis: ECV Quantification by CT in TAVR Patients

• Published in: JACC. Cardiovascular imaging Vol. 13; no. 10; pp. 2177 - 2189
• Main Authors: Scully, Paul R, Patel, Kush P, Saberwal, Bunny, Klotz, Ernst, Augusto, João B, Thornton, George D, Hughes, Rebecca K, Manisty, Charlotte, Lloyd, Guy, Newton, James D, Sabharwal, Nikant, Kelion, Andrew, Kennon, Simon, Ozkor, Muhiddin, Mullen, Michael, Hartman, Neil, Cavalcante, João L, Menezes, Leon J, Hawkins, Philip N, Treibel, Thomas A, Moon, James C, Pugliese, Francesca
• Format: Journal Article
• Language: English
• Published: United States Elsevier 01.10.2020
• Subjects: Aged, 80 and over; Amyloidosis; Aortic Valve Stenosis - surgery; Female; Humans; Male; Original Research; Predictive Value of Tests; Stroke Volume; Tomography, X-Ray Computed; Transcatheter Aortic Valve Replacement; Treatment Outcome; Ventricular Function, Left; computed tomography; cardiac amyloidosis; aortic stenosis; extracellular volume
• ISSN: 1876-7591; 1936-878X; 1876-7591
• DOI: 10.1016/j.jcmg.2020.05.029

The purpose of this study was to validate computed tomography measured ECV (ECV ) as part of routine evaluation for the detection of cardiac amyloid in patients with aortic stenosis (AS)-amyloid. AS-amyloid affects 1 in 7 elderly patients referred for transcatheter aortic valve replacement (TAVR). Bone scintigraphy with exclusion of a plasma cell dyscrasia can diagnose transthyretin-related cardiac amyloid noninvasively, for which novel treatments are emerging. Amyloid interstitial expansion increases the myocardial extracellular volume (ECV). Patients with severe AS underwent bone scintigraphy (Perugini grade 0, negative; Perugini grades 1 to 3, increasingly positive) and routine TAVR evaluation CT imaging with ECV using 3- and 5-min post-contrast acquisitions. Twenty non-AS control patients also had ECV performed using the 5-min post-contrast acquisition. A total of 109 patients (43% male; mean age 86 ± 5 years) with severe AS and 20 control subjects were recruited. Sixteen (15%) had AS-amyloid on bone scintigraphy (grade 1, n = 5; grade 2, n = 11). ECV was 32 ± 3%, 34 ± 4%, and 43 ± 6% in Perugini grades 0, 1, and 2, respectively (p < 0.001 for trend) with control subjects lower than lone AS (28 ± 2%; p < 0.001). ECV accuracy for AS-amyloid detection versus lone AS was 0.87 (0.95 for Tc-3,3-diphosphono-1,2-propanodicarboxylic acid Perugini grade 2 only), outperforming conventional electrocardiogram and echocardiography parameters. One composite parameter, the voltage/mass ratio, had utility (similar AUC of 0.87 for any cardiac amyloid detection), although in one-third of patients, this could not be calculated due to bundle branch block or ventricular paced rhythm. ECV during routine CT TAVR evaluation can reliably detect AS-amyloid, and the measured ECV tracks the degree of infiltration. Another measure of interstitial expansion, the voltage/mass ratio, also performed well.