2021 Volume 85 Issue 10 Pages 1894-
A 79-year-old woman with positive anti-centromere antibody was admitted for heart failure. As compared with images taken 2 years prior (Figure A,D), newly developed mitral and tricuspid valvular thickening (Figure E) were found and Libman-Sacks endocarditis was suspected; 3 months later, monoclonal gammopathy with Bence-Jones protein was identified. Amyloid light-chain cardiac amyloidosis (ALCA) with multiple myeloma was diagnosed by bone and cardiac biopsies (Supplementary Figure A). Review of the echocardiograms revealed left atrial wall thickening (LAWT) (Figure B). At 4 months after the ALCA diagnosis, ventricular wall thickening occurred (Figure C,F). Along with these changes, progressive decrease in both A-wave velocity and QRS voltage was observed (Figure G–L). Additionally, cardiac magnetic resonance images demonstrated late gadolinium enhancement only in the left atrium, suggesting minimal damage in the left ventricle (Supplementary Figure B). She underwent chemotherapy, but died within 1 year.
Echocardiography in the parasternal long-axis view (A–C), apical 4-chamber view (D–F), transmitral flow velocity pattern (G–I), and ECG of limb leads (J–L). Images in the left, middle, and right columns were taken 2 years before, 3 months before, and 4 months after, respectively, the diagnosis of amyloid light-chain cardiac amyloidosis.
ALCA is induced by the deposition of misfolded amyloid light-chain proteins in the heart, typically presenting as valve and ventricular thickening.1 However, an ALCA diagnosis in an aged patient with suspected scleroderma is challenging because the thickened valves resemble aged degeneration or Libman-Sacks endocarditis.
LAWT is a rare feature to suggest ALCA. While its standardization is still under development, strain imaging can help identify atrial functional impairment.2
This case highlights that LAWT is crucial for the early detection of ALCA, especially when valvular or ventricular findings are indecisive.
Y.K. is a member of Circulation Journal’s Editorial Team.
Please find supplementary file(s);
http://dx.doi.org/10.1253/circj.CJ-21-0485