PMID- 31189919
OWN - NLM
STAT- MEDLINE
DCOM- 20201014
LR  - 20210109
IS  - 2045-2322 (Electronic)
IS  - 2045-2322 (Linking)
VI  - 9
IP  - 1
DP  - 2019 Jun 12
TI  - Predictors of Mortality in Light Chain Cardiac Amyloidosis with Heart Failure.
PG  - 8552
LID - 10.1038/s41598-019-44912-x [doi]
LID - 8552
AB  - Cardiac involvement in systemic amyloidosis (AL) occurs in ~50% of all AL 
      patients. However once symptomatic heart failure develops, therapeutic options 
      are limited thereby conferring a poor overall prognosis. The median survival is 
      <6 months when AL patients are untreated for the underlying plasma cell 
      dyscrasia. We thus sought to identify risk factors of increased mortality in 
      treatment-naive, AL cardiac amyloidosis with heart failure. Patients with 
      biopsy-proven AL cardiac amyloid, who presented with heart failure and did not 
      received prior AL treatment, were enrolled between 2004-2014, at the initial 
      visit to the Amyloidosis Center at Boston University Medical Center. Routine 
      laboratory tests, physical examination and echocardiography data were collected. 
      There were 165 predominantly white (76.4%), and male (61%) patients, with a mean 
      age of 61.6 +/- 9.5 years. Median survival was 10.9 months (95% CI 6.2-14.7). By 
      multivariate analysis increased relative wall thickness (RWT) [HR 6.70; 95% CI 
      2.45-18.30), older age (HR 1.04; 95% CI 1.01-1.06), higher New York Heart 
      Association (NYHA) functional class (HR 1.50; 95% CI 1.02-2.2), log brain 
      natriuretic peptide (BNP) levels (HR 1.45; 95% CI 1.15-1.81) and C-reactive 
      protein (CRP) levels (HR 1.02; 95% CI 1.00-1.04) were significant predictors for 
      increased mortality. In conclusion, in treatment-naive, AL cardiac amyloidosis 
      patients with heart failure symptoms who lack these high-risk features may have a 
      better outcome. These findings might allow for better risk stratification 
      although outcomes are still poor.
FAU - Tahir, Usman A
AU  - Tahir UA
AD  - Evans Department of Medicine, Boston University School of Medicine and Boston 
      Medical Center, Boston, USA.
FAU - Doros, Gheorghe
AU  - Doros G
AD  - Department of Biostatistics, Boston University School of Public Health, Boston, 
      USA.
FAU - Kim, John S
AU  - Kim JS
AD  - Evans Department of Medicine, Boston University School of Medicine and Boston 
      Medical Center, Boston, USA.
FAU - Connors, Lawreen H
AU  - Connors LH
AD  - Alan and Sandra Gerry Amyloid Research Laboratory in the Amyloidosis Center, 
      Boston University School of Medicine, Boston, USA.
FAU - Seldin, David C
AU  - Seldin DC
AD  - Evans Department of Medicine, Boston University School of Medicine and Boston 
      Medical Center, Boston, USA.
AD  - Alan and Sandra Gerry Amyloid Research Laboratory in the Amyloidosis Center, 
      Boston University School of Medicine, Boston, USA.
FAU - Sam, Flora
AU  - Sam F
AD  - Whitaker Cardiovascular Institute, Boston University School of Medicine, Boston, 
      USA. flora.sam@bmc.org.
AD  - Cardiovascular Section, Boston University School of Medicine, Boston, USA. 
      flora.sam@bmc.org.
AD  - Evans Department of Medicine, Boston University School of Medicine and Boston 
      Medical Center, Boston, USA. flora.sam@bmc.org.
LA  - eng
GR  - R01 HL117153/HL/NHLBI NIH HHS/United States
PT  - Clinical Trial
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, Non-U.S. Gov't
DEP - 20190612
PL  - England
TA  - Sci Rep
JT  - Scientific reports
JID - 101563288
RN  - 0 (Biomarkers)
RN  - 114471-18-0 (Natriuretic Peptide, Brain)
RN  - 9007-41-4 (C-Reactive Protein)
SB  - IM
MH  - Aged
MH  - Biomarkers/blood
MH  - C-Reactive Protein/*metabolism
MH  - Disease-Free Survival
MH  - *Echocardiography
MH  - Female
MH  - *Heart Failure/blood/diagnostic imaging/mortality/therapy
MH  - Humans
MH  - *Immunoglobulin Light-chain Amyloidosis/blood/diagnostic 
      imaging/mortality/therapy
MH  - Male
MH  - Middle Aged
MH  - Natriuretic Peptide, Brain/*blood
MH  - Survival Rate
PMC - PMC6561903
COIS- The authors declare no competing interests.
EDAT- 2019/06/14 06:00
MHDA- 2020/10/21 06:00
PMCR- 2019/06/12
CRDT- 2019/06/14 06:00
PHST- 2018/12/31 00:00 [received]
PHST- 2019/05/28 00:00 [accepted]
PHST- 2019/06/14 06:00 [entrez]
PHST- 2019/06/14 06:00 [pubmed]
PHST- 2020/10/21 06:00 [medline]
PHST- 2019/06/12 00:00 [pmc-release]
AID - 10.1038/s41598-019-44912-x [pii]
AID - 44912 [pii]
AID - 10.1038/s41598-019-44912-x [doi]
PST - epublish
SO  - Sci Rep. 2019 Jun 12;9(1):8552. doi: 10.1038/s41598-019-44912-x.