Thibaud Damy1,2,3,4,5,6, Arnaud Jaccard7,8, Aziz Guellich1,2,3,4, David Lavergne7,8, Arnault Galat1,2,3,4, Jean-François Deux1,2,3,9, Luc Hittinger1,2,3,4, Jehan Dupuis2,4,5,10, Valérie Frenkel2,3,11, Charlotte Rigaud7,8, Violaine Plante-Bordeneuve2,3,4,5,6,12, Diane Bodez1,2,3,4, Dania Mohty7,8. 1. a Department of Cardiology , AP-HP, Henri Mondor Teaching Hospital , Créteil , France. 2. b University Paris-East (UPEC) School of Medicine , Créteil , France. 3. c GRC Amyloid Research Institute, IMRB INSERM U955 , Créteil , France. 4. d Mondor Amyloidosis Network, Henri Mondor Teaching Hospital , Créteil , France. 5. e AP-HP, INSERM, Clinical Investigations Center 1430, Henri Mondor Teaching Hospital , Créteil , France. 6. f Department of Clinical Research and Public Health , Clinical Investigations Center 006, Henri Mondor Teaching Hospital , Créteil , France. 7. g Department of Cardiology , Dupuytren Teaching Hospital , Limoges , France. 8. h Dupuytren Teaching Hospital, AL Amyloidosis Referral Center , Limoges , France. 9. i Department of Radiology , AP-HP, Henri Mondor Teaching Hospital , Créteil , France. 10. j Department of Hematology , AP-HP, Henri Mondor Teaching Hospital , Créteil , France. 11. l Department of Immunohematolgy , AP-HP, Henri Mondor Teaching Hospital , Créteil , France. 12. k Department of Neurology , AP-HP, Henri Mondor Teaching Hospital , Créteil , France , and.
Abstract
BACKGROUND: The prognosis of amyloidosis is known to depend heavily on cardiac function and may be improved by identifying patients at highest risk for adverse cardiac events. AIMS: Identify predictors of mortality in patients with cardiac light-chain amyloidosis (AL), hereditary transthyretin amyloidosis (m-TTR), or wild-type transthyretin amyloidosis (WT-TTR) to prompt physician to refer these patients to dedicated centers. METHODS AND RESULTS: Observational study. About 266 patients referred for suspected cardiac amyloidosis (CA) in two French university centers were included. About 198 patients had CA (AL = 118, m-TTR = 57, and WT-TTR = 23). Their median (25th-75th percentile) age, NT-proBNP left ventricular ejection fraction were, respectively, 68 years (59-76), 2339 pg mL-1 (424-5974), and 60% (48-66). About 31% were in NYHA class III-IV. Interventricular septal thickness was greater in the m-TTR and WT-TTR groups than in the AL group (p < 0.0001). Median follow-up in survivor was 26 months (15-44) and 87 (44%) patients died. By multivariate analysis, independent predictors of mortality for AL amyloidosis were the following: age, cardiac output and NT-proBNP; for TTR amyloidosis was: NT-proBNP. When all amyloidosis were combined NT-proBNP, low cardiac output and pericardial effusion were independently associated with mortality. CONCLUSION: NT-proBNP is a strong prognosticator in the three types of cardiac amyloidosis. High NT-proBNP, low cardiac output, and pericardial effusion at the time of screening should prompt physician to refer the patients to amyloidosis referral center.
BACKGROUND: The prognosis of amyloidosis is known to depend heavily on cardiac function and may be improved by identifying patients at highest risk for adverse cardiac events. AIMS: Identify predictors of mortality in patients with cardiac light-chain amyloidosis (AL), hereditary transthyretin amyloidosis (m-TTR), or wild-type transthyretin amyloidosis (WT-TTR) to prompt physician to refer these patients to dedicated centers. METHODS AND RESULTS: Observational study. About 266 patients referred for suspected cardiac amyloidosis (CA) in two French university centers were included. About 198 patients had CA (AL = 118, m-TTR = 57, and WT-TTR = 23). Their median (25th-75th percentile) age, NT-proBNP left ventricular ejection fraction were, respectively, 68 years (59-76), 2339 pg mL-1 (424-5974), and 60% (48-66). About 31% were in NYHA class III-IV. Interventricular septal thickness was greater in the m-TTR and WT-TTR groups than in the AL group (p < 0.0001). Median follow-up in survivor was 26 months (15-44) and 87 (44%) patients died. By multivariate analysis, independent predictors of mortality for AL amyloidosis were the following: age, cardiac output and NT-proBNP; for TTRamyloidosis was: NT-proBNP. When all amyloidosis were combined NT-proBNP, low cardiac output and pericardial effusion were independently associated with mortality. CONCLUSION: NT-proBNP is a strong prognosticator in the three types of cardiac amyloidosis. High NT-proBNP, low cardiac output, and pericardial effusion at the time of screening should prompt physician to refer the patients to amyloidosis referral center.
Authors: Sharmila Dorbala; Yukio Ando; Sabahat Bokhari; Angela Dispenzieri; Rodney H Falk; Victor A Ferrari; Marianna Fontana; Olivier Gheysens; Julian D Gillmore; Andor W J M Glaudemans; Mazen A Hanna; Bouke P C Hazenberg; Arnt V Kristen; Raymond Y Kwong; Mathew S Maurer; Giampaolo Merlini; Edward J Miller; James C Moon; Venkatesh L Murthy; C Cristina Quarta; Claudio Rapezzi; Frederick L Ruberg; Sanjiv J Shah; Riemer H J A Slart; Hein J Verberne; Jamieson M Bourque Journal: J Nucl Cardiol Date: 2019-12 Impact factor: 5.952