PMID- 27364045
OWN - NLM
STAT- MEDLINE
DCOM- 20170626
LR  - 20220331
IS  - 1558-3597 (Electronic)
IS  - 0735-1097 (Linking)
VI  - 68
IP  - 1
DP  - 2016 Jul 5
TI  - Cardiac Amyloid Load: A Prognostic and Predictive Biomarker in Patients With 
      Light-Chain Amyloidosis.
PG  - 13-24
LID - S0735-1097(16)32998-9 [pii]
LID - 10.1016/j.jacc.2016.04.035 [doi]
AB  - BACKGROUND: Cardiac amyloid load has not been analyzed for its effect on 
      mortality in patients with amyloid light-chain (AL) cardiac amyloidosis. 
      OBJECTIVES: This study retrospectively compared histological amyloid load with 
      common clinical predictors of mortality. METHODS: This study assessed 216 
      patients with histologically confirmed cardiac amyloidosis at a single center 
      with electrocardiography, echocardiography, and laboratory testing. RESULTS: 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. CONCLUSIONS: 
      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.
CI  - Copyright (c) 2016 American College of Cardiology Foundation. Published by Elsevier 
      Inc. All rights reserved.
FAU - Kristen, Arnt V
AU  - Kristen AV
AD  - Department of Cardiology, Angiology, and Respiratory Medicine, University of 
      Heidelberg, Heidelberg, Germany. Electronic address: 
      Arnt.Kristen@med.uni-heidelberg.de.
FAU - Brokbals, Eva
AU  - Brokbals E
AD  - Department of Cardiology, Angiology, and Respiratory Medicine, University of 
      Heidelberg, Heidelberg, Germany.
FAU - Aus dem Siepen, Fabian
AU  - Aus dem Siepen F
AD  - Department of Cardiology, Angiology, and Respiratory Medicine, University of 
      Heidelberg, Heidelberg, Germany.
FAU - Bauer, Ralf
AU  - Bauer R
AD  - Department of Cardiology, Angiology, and Respiratory Medicine, University of 
      Heidelberg, Heidelberg, Germany.
FAU - Hein, Selina
AU  - Hein S
AD  - Department of Cardiology, Angiology, and Respiratory Medicine, University of 
      Heidelberg, Heidelberg, Germany.
FAU - Aurich, Matthias
AU  - Aurich M
AD  - Department of Cardiology, Angiology, and Respiratory Medicine, University of 
      Heidelberg, Heidelberg, Germany.
FAU - Riffel, Johannes
AU  - Riffel J
AD  - Department of Cardiology, Angiology, and Respiratory Medicine, University of 
      Heidelberg, Heidelberg, Germany.
FAU - Behrens, Hans-Michael
AU  - Behrens HM
AD  - Institute of Pathology, Christian-Albrechts-University, Kiel, Germany.
FAU - Kruger, Sandra
AU  - Kruger S
AD  - Institute of Pathology, Christian-Albrechts-University, Kiel, Germany.
FAU - Schirmacher, Peter
AU  - Schirmacher P
AD  - Institute of Pathology, University of Heidelberg, Heidelberg, Germany.
FAU - Katus, Hugo A
AU  - Katus HA
AD  - Department of Cardiology, Angiology, and Respiratory Medicine, University of 
      Heidelberg, Heidelberg, Germany; DZHK (German Center for Cardiovascular 
      Research), Site Heidelberg/Mannheim, Heidelberg, Germany.
FAU - Rocken, Christoph
AU  - Rocken C
AD  - Institute of Pathology, Christian-Albrechts-University, Kiel, Germany.
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - J Am Coll Cardiol
JT  - Journal of the American College of Cardiology
JID - 8301365
RN  - 0 (Amyloid)
RN  - 0 (Biomarkers)
SB  - IM
CIN - J Am Coll Cardiol. 2016 Jul 5;68(1):25-8. PMID: 27364046
CIN - J Am Coll Cardiol. 2016 Dec 6;68(22):2493-2494. PMID: 27908360
CIN - J Am Coll Cardiol. 2016 Dec 6;68(22):2494-2495. PMID: 27908361
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Amyloid/*analysis/*metabolism
MH  - Amyloidosis/*metabolism/mortality/pathology
MH  - Biomarkers/metabolism
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Myocardium/*chemistry/metabolism/pathology
MH  - Predictive Value of Tests
MH  - Prognosis
MH  - Retrospective Studies
OTO - NOTNLM
OT  - amyloidosis
OT  - endomyocardial biopsy
OT  - immunohistochemistry
OT  - light-chain amyloid
OT  - survival
OT  - transthyretin
EDAT- 2016/07/02 06:00
MHDA- 2017/06/27 06:00
CRDT- 2016/07/02 06:00
PHST- 2015/11/23 00:00 [received]
PHST- 2016/04/03 00:00 [revised]
PHST- 2016/04/12 00:00 [accepted]
PHST- 2016/07/02 06:00 [entrez]
PHST- 2016/07/02 06:00 [pubmed]
PHST- 2017/06/27 06:00 [medline]
AID - S0735-1097(16)32998-9 [pii]
AID - 10.1016/j.jacc.2016.04.035 [doi]
PST - ppublish
SO  - J Am Coll Cardiol. 2016 Jul 5;68(1):13-24. doi: 10.1016/j.jacc.2016.04.035.