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- Title
Quantification of right ventricular amyloid burden with <sup>18</sup>F-florbetapir positron emission tomography/computed tomography and its association with right ventricular dysfunction and outcomes in light-chain amyloidosis.
- Authors
Datar, Yesh; Clerc, Olivier F; Cuddy, Sarah A M; Kim, Sirwoo; Taylor, Alexandra; Neri, Jocelyn Canseco; Benz, Dominik C; Bianchi, Giada; Yee, Andrew J; Sanchorawala, Vaishali; Ruberg, Frederick L; Landau, Heather; Liao, Ronglih; Kijewski, Marie Foley; Jerosch-Herold, Michael; Kwong, Raymond Y; Carli, Marcelo F Di; Falk, Rodney H; Dorbala, Sharmila
- Abstract
Aims In systemic light-chain (AL) amyloidosis, quantification of right ventricular (RV) amyloid burden has been limited and the pathogenesis of RV dysfunction is poorly understood. Using 18F-florbetapir positron emission tomography/computed tomography (PET/CT), we aimed to quantify RV amyloid; correlate RV amyloid with RV structure and function; determine the independent contributions of RV, left ventricular (LV), and lung amyloid to RV function; and associate RV amyloid with major adverse cardiac events (MACE: death, heart failure hospitalization, cardiac transplantation). Methods and results We prospectively enrolled 106 participants with AL amyloidosis (median age 62 years, 55% males) who underwent 18F-florbetapir PET/CT, magnetic resonance imaging, and echocardiography. 18F-florbetapir PET/CT identified RV amyloid in 63% of those with and 40% of those without cardiac involvement by conventional criteria. RV amyloid burden correlated with RV ejection fraction (EF), RV free wall longitudinal strain (FWLS), RV wall thickness, RV mass index, N-terminal pro-brain natriuretic peptide, troponin T, LV amyloid, and lung amyloid (each P < 0.001). In multivariable analysis, RV amyloid burden, but not LV or lung amyloid burden, predicted RV dysfunction (EF P = 0.014; FWLS P < 0.001). During a median follow-up of 28 months, RV amyloid burden predicted MACE (P < 0.001). Conclusion This study shows for the first time that 18F-florbetapir PET/CT identifies early RV amyloid in systemic AL amyloidosis prior to alterations in RV structure and function. Increasing RV amyloid on 18F-florbetapir PET/CT is associated with worse RV structure and function, predicts RV dysfunction, and predicts MACE. These results imply a central role for RV amyloid in the pathogenesis of RV dysfunction.
- Subjects
CARDIAC amyloidosis; IMMUNOGLOBULIN light chains; CARDIOMYOPATHIES; RESEARCH funding; COMPUTED tomography; TREATMENT effectiveness; DESCRIPTIVE statistics; PEPTIDE hormones; MULTIVARIATE analysis; AMYLOIDOSIS; LUNGS; LONGITUDINAL method; RIGHT ventricular dysfunction; EMISSION-computed tomography; RIGHT heart ventricle; ECHOCARDIOGRAPHY; EVALUATION
- Publication
European Heart Journal - Cardiovascular Imaging, 2024, Vol 25, Issue 5, p687
- ISSN
2047-2404
- Publication type
Article
- DOI
10.1093/ehjci/jead350