{"doi":"10.1093/ehjci/jead350","title":"Quantification of right ventricular amyloid burden with 18F-florbetapir positron emission tomography/computed tomography and its association with right ventricular dysfunction and outcomes in light-chain amyloidosis","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.","journal":"European Heart Journal - Cardiovascular Imaging","year":2024,"id":429833,"datarank":0.47032413238937254,"base_score":3.1354942159291497,"endowment":3.1354942159291497,"self_citation_contribution":0.47032413238937254,"citation_network_contribution":0.0,"self_endowment_contribution":0.47032413238937254,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":22,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9548,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2024-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":830905,"name":"Olivier Clerc","orcid":"0000-0001-7725-9318","position":1,"is_corresponding":false},{"id":299760,"name":"Sarah Cuddy","orcid":"0000-0002-2124-2151","position":2,"is_corresponding":false},{"id":537010,"name":"Sirwoo Kim","orcid":null,"position":3,"is_corresponding":false},{"id":1173723,"name":"Alexandra A. 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Ruberg","orcid":"0000-0002-6424-4413","position":10,"is_corresponding":false},{"id":298161,"name":"Heather Landau","orcid":"0000-0002-3152-1189","position":11,"is_corresponding":false},{"id":299767,"name":"Ronglih Liao","orcid":"0000-0002-9598-9020","position":12,"is_corresponding":false},{"id":299763,"name":"Marie Foley Kijewski","orcid":"0000-0001-8365-1447","position":13,"is_corresponding":false},{"id":225212,"name":"Michael Jerosch‐Herold","orcid":"0000-0001-9018-6731","position":14,"is_corresponding":false},{"id":232181,"name":"Raymond Y. Kwong","orcid":"0000-0001-8212-0759","position":15,"is_corresponding":false},{"id":260674,"name":"Marcelo F. Di Carli","orcid":"0000-0003-3119-025X","position":16,"is_corresponding":false},{"id":299762,"name":"Rodney H. 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