title: Cardiac Amyloid Load: A Prognostic and Predictive Biomarker in Patients With Light-Chain Amyloidosis.
abstract: Cardiac amyloid load has not been analyzed for its effect on mortality in patients with amyloid light-chain (AL) cardiac amyloidosis.
  
  
  
  This study retrospectively compared histological amyloid load with common clinical predictors of mortality.
  
  
  
  This study assessed 216 patients with histologically confirmed cardiac amyloidosis at a single center with electrocardiography, echocardiography, and laboratory testing.
  
  
  
  AL amyloid deposits were usually distributed in a reticular/pericellular pattern, whereas transthyretin amyloid (ATTR) more commonly showed patchy deposits. Median amyloid load was 30.5%; no amyloid load was above 70%. During follow-up (median 19.1 months), 112 patients died. Chemotherapy had a significant effect on overall survival in AL amyloidosis (16.2 months vs. 1.4 months; p = 0.003). Patients with <20% AL amyloid load who responded to chemotherapy showed significantly better survival than nonresponders. According to univariate analysis, predictors of survival in AL amyloidosis included sex, Karnofsky index, New York Heart Association (NYHA) functional class, diastolic blood pressure, estimated glomerular filtration rate, N-terminal pro-B-type natriuretic peptide, mineralocorticoid receptor antagonists, low voltage, ineligibility for chemotherapy, response to chemotherapy, and amyloid load. Independent predictors of mortality by multivariate analysis included NYHA functional class (III vs. II), estimated glomerular filtration rate, responders to chemotherapy, and amyloid load. In ATTR amyloidosis, survival correlated with NYHA functional class, diastolic blood pressure, and use of diuretic agents. Following Cox regression analysis, NYHA functional class (III vs. II; p < 0.05) remained the only independent predictor of patient survival in ATTR amyloidosis.
  
  
  
  Early identification of subjects with AL amyloid is essential given that in late-stage disease with extensive amyloid load, our data suggested that outcomes are not affected by administration of chemotherapy.
authors.affiliation: Department of Cardiology, Angiology, and Respiratory Medicine, University of Heidelberg, Heidelberg, Germany. Electronic address: Arnt.Kristen@med.uni-heidelberg.de.
authors.forename: Arnt V
authors.initials: AV
authors.lastname: Kristen
authors.affiliation: Department of Cardiology, Angiology, and Respiratory Medicine, University of Heidelberg, Heidelberg, Germany.
authors.forename: Eva
authors.initials: E
authors.lastname: Brokbals
authors.affiliation: Department of Cardiology, Angiology, and Respiratory Medicine, University of Heidelberg, Heidelberg, Germany.
authors.forename: Fabian
authors.initials: F
authors.lastname: Aus dem Siepen
authors.affiliation: Department of Cardiology, Angiology, and Respiratory Medicine, University of Heidelberg, Heidelberg, Germany.
authors.forename: Ralf
authors.initials: R
authors.lastname: Bauer
authors.affiliation: Department of Cardiology, Angiology, and Respiratory Medicine, University of Heidelberg, Heidelberg, Germany.
authors.forename: Selina
authors.initials: S
authors.lastname: Hein
authors.affiliation: Department of Cardiology, Angiology, and Respiratory Medicine, University of Heidelberg, Heidelberg, Germany.
authors.forename: Matthias
authors.initials: M
authors.lastname: Aurich
authors.affiliation: Department of Cardiology, Angiology, and Respiratory Medicine, University of Heidelberg, Heidelberg, Germany.
authors.forename: Johannes
authors.initials: J
authors.lastname: Riffel
authors.affiliation: Institute of Pathology, Christian-Albrechts-University, Kiel, Germany.
authors.forename: Hans-Michael
authors.initials: HM
authors.lastname: Behrens
authors.affiliation: Institute of Pathology, Christian-Albrechts-University, Kiel, Germany.
authors.forename: Sandra
authors.initials: S
authors.lastname: Krüger
authors.affiliation: Institute of Pathology, University of Heidelberg, Heidelberg, Germany.
authors.forename: Peter
authors.initials: P
authors.lastname: Schirmacher
authors.affiliation: Department of Cardiology, Angiology, and Respiratory Medicine, University of Heidelberg, Heidelberg, Germany; DZHK (German Center for Cardiovascular Research), Site Heidelberg/Mannheim, Heidelberg, Germany.
authors.forename: Hugo A
authors.initials: HA
authors.lastname: Katus
authors.affiliation: Institute of Pathology, Christian-Albrechts-University, Kiel, Germany.
authors.forename: Christoph
authors.initials: C
authors.lastname: Röcken
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000682:Amyloid / Q000032:analysis* / Q000378:metabolism*; D000686:Amyloidosis / Q000378:metabolism* / Q000401:mortality / Q000473:pathology; D015415:Biomarkers / Q000378:metabolism; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009206:Myocardium / Q000737:chemistry* / Q000378:metabolism / Q000473:pathology; D011237:Predictive Value of Tests; D011379:Prognosis; D012189:Retrospective Studies
chemical_list: D000682:Amyloid; D015415:Biomarkers
keywords: amyloidosis; endomyocardial biopsy; immunohistochemistry; light-chain amyloid; survival; transthyretin