title: Identification of prognostic markers in transthyretin and AL cardiac amyloidosis.
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.
  
  
  OBJECTIVE
  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.
  
  
  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.
  
  
  CONCLUSIONS
  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.affiliation: a Department of Cardiology , AP-HP, Henri Mondor Teaching Hospital , Créteil , France.|b University Paris-East (UPEC) School of Medicine , Créteil , France.|c GRC Amyloid Research Institute, IMRB INSERM U955 , Créteil , France.|d Mondor Amyloidosis Network, Henri Mondor Teaching Hospital , Créteil , France.|e AP-HP, INSERM, Clinical Investigations Center 1430, Henri Mondor Teaching Hospital , Créteil , France.|f Department of Clinical Research and Public Health , Clinical Investigations Center 006, Henri Mondor Teaching Hospital , Créteil , France.
authors.forename: Thibaud
authors.initials: T
authors.lastname: Damy
authors.affiliation: g Department of Cardiology , Dupuytren Teaching Hospital , Limoges , France.|h Dupuytren Teaching Hospital, AL Amyloidosis Referral Center , Limoges , France.
authors.forename: Arnaud
authors.initials: A
authors.lastname: Jaccard
authors.affiliation: a Department of Cardiology , AP-HP, Henri Mondor Teaching Hospital , Créteil , France.|b University Paris-East (UPEC) School of Medicine , Créteil , France.|c GRC Amyloid Research Institute, IMRB INSERM U955 , Créteil , France.|d Mondor Amyloidosis Network, Henri Mondor Teaching Hospital , Créteil , France.
authors.forename: Aziz
authors.initials: A
authors.lastname: Guellich
authors.affiliation: g Department of Cardiology , Dupuytren Teaching Hospital , Limoges , France.|h Dupuytren Teaching Hospital, AL Amyloidosis Referral Center , Limoges , France.
authors.forename: David
authors.initials: D
authors.lastname: Lavergne
authors.affiliation: a Department of Cardiology , AP-HP, Henri Mondor Teaching Hospital , Créteil , France.|b University Paris-East (UPEC) School of Medicine , Créteil , France.|c GRC Amyloid Research Institute, IMRB INSERM U955 , Créteil , France.|d Mondor Amyloidosis Network, Henri Mondor Teaching Hospital , Créteil , France.
authors.forename: Arnault
authors.initials: A
authors.lastname: Galat
authors.affiliation: a Department of Cardiology , AP-HP, Henri Mondor Teaching Hospital , Créteil , France.|b University Paris-East (UPEC) School of Medicine , Créteil , France.|c GRC Amyloid Research Institute, IMRB INSERM U955 , Créteil , France.|i Department of Radiology , AP-HP, Henri Mondor Teaching Hospital , Créteil , France.
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Deux
authors.affiliation: a Department of Cardiology , AP-HP, Henri Mondor Teaching Hospital , Créteil , France.|b University Paris-East (UPEC) School of Medicine , Créteil , France.|c GRC Amyloid Research Institute, IMRB INSERM U955 , Créteil , France.|d Mondor Amyloidosis Network, Henri Mondor Teaching Hospital , Créteil , France.
authors.forename: Luc
authors.initials: L
authors.lastname: Hittinger
authors.affiliation: b University Paris-East (UPEC) School of Medicine , Créteil , France.|d Mondor Amyloidosis Network, Henri Mondor Teaching Hospital , Créteil , France.|e AP-HP, INSERM, Clinical Investigations Center 1430, Henri Mondor Teaching Hospital , Créteil , France.|j Department of Hematology , AP-HP, Henri Mondor Teaching Hospital , Créteil , France.
authors.forename: Jehan
authors.initials: J
authors.lastname: Dupuis
authors.affiliation: b University Paris-East (UPEC) School of Medicine , Créteil , France.|c GRC Amyloid Research Institute, IMRB INSERM U955 , Créteil , France.|l Department of Immunohematolgy , AP-HP, Henri Mondor Teaching Hospital , Créteil , France.
authors.forename: Valérie
authors.initials: V
authors.lastname: Frenkel
authors.affiliation: g Department of Cardiology , Dupuytren Teaching Hospital , Limoges , France.|h Dupuytren Teaching Hospital, AL Amyloidosis Referral Center , Limoges , France.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Rigaud
authors.affiliation: b University Paris-East (UPEC) School of Medicine , Créteil , France.|c GRC Amyloid Research Institute, IMRB INSERM U955 , Créteil , France.|d Mondor Amyloidosis Network, Henri Mondor Teaching Hospital , Créteil , France.|e AP-HP, INSERM, Clinical Investigations Center 1430, Henri Mondor Teaching Hospital , Créteil , France.|f Department of Clinical Research and Public Health , Clinical Investigations Center 006, Henri Mondor Teaching Hospital , Créteil , France.|k Department of Neurology , AP-HP, Henri Mondor Teaching Hospital , Créteil , France , and.
authors.forename: Violaine
authors.initials: V
authors.lastname: Plante-Bordeneuve
authors.affiliation: a Department of Cardiology , AP-HP, Henri Mondor Teaching Hospital , Créteil , France.|b University Paris-East (UPEC) School of Medicine , Créteil , France.|c GRC Amyloid Research Institute, IMRB INSERM U955 , Créteil , France.|d Mondor Amyloidosis Network, Henri Mondor Teaching Hospital , Créteil , France.
authors.forename: Diane
authors.initials: D
authors.lastname: Bodez
authors.affiliation: g Department of Cardiology , Dupuytren Teaching Hospital , Limoges , France.|h Dupuytren Teaching Hospital, AL Amyloidosis Referral Center , Limoges , France.
authors.forename: Dania
authors.initials: D
authors.lastname: Mohty
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D015415:Biomarkers / Q000032:analysis; D002303:Cardiac Output, Low / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D009202:Cardiomyopathies / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D010490:Pericardial Effusion / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000503:physiopathology; D011228:Prealbumin / Q000378:metabolism; D011379:Prognosis; D013318:Stroke Volume; D016019:Survival Analysis; D016277:Ventricular Function, Left
chemical_list: D015415:Biomarkers; D007147:Immunoglobulin Light Chains; D010446:Peptide Fragments; D011228:Prealbumin; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain
keywords: Amyloidosis; brain natriuretic peptide; echocardiography; heart failure; infiltrative cardiomyopathy; prognosis