Identification of Prognostic Markers in Transthyretin and AL Cardiac Amyloidosis | oneAMYLOIDOSISvoice
Scientific Articles

Identification of Prognostic Markers in Transthyretin and AL Cardiac Amyloidosis

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source: Amyloid: The International Journal of Experimental and Clinical Investigation

year: 2016

authors: Damy T, Jaccard A, Guellich A, Lavergne D, Galat A, Deux JF, Hittinger L, Dupuis J, Frenkel V, Rigaud C, Plante-Bordeneuve V, Bodez D, Mohty D



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.


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.


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.

organization: Henri Mondor Teaching Hospital, France; University Paris-East (UPEC) School of Medicine, France; GRC Amyloid Research Institute, France; Dupuytren Teaching Hospital, France; AL Amyloidosis Referral Center, France

DOI: 10.1080/13506129.2016.1221815

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