Identifying Cardiac Amyloid in Aortic Stenosis

• Published in: JACC. Cardiovascular imaging 2020-10, Vol.13 (10), p.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: Article
• Language: English
• Subjects: aortic stenosis; cardiac amyloidosis; computed tomography; extracellular volume
• ISSN: 1936-878X; 1876-7591
• DOI: 10.1016/j.jcmg.2020.05.029

The purpose of this study was to validate computed tomography measured ECV (ECVCT) 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 ECVCT using 3- and 5-min post-contrast acquisitions. Twenty non-AS control patients also had ECVCT 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). ECVCT was 32 ± 3%, 34 ± 4%, and 43 ± 6% in Perugini grades 0, 1, and 2, respectively (p