PMID- 27339491
OWN - NLM
STAT- MEDLINE
DCOM- 20170626
LR  - 20181002
IS  - 1558-3597 (Electronic)
IS  - 0735-1097 (Linking)
VI  - 67
IP  - 25
DP  - 2016 Jun 28
TI  - Efficacy of Chemotherapy for Light-Chain Amyloidosis in Patients Presenting With 
      Symptomatic Heart Failure.
PG  - 2941-8
LID - S0735-1097(16)32928-X [pii]
LID - 10.1016/j.jacc.2016.03.593 [doi]
AB  - BACKGROUND: Light-chain amyloidosis (AL) with cardiac involvement carries a poor 
      prognosis; median untreated survival is <6 months. Three-drug therapy with 
      bortezomib, dexamethasone, and an alkylating agent (BDex+AA) is associated with 
      improved biomarker response rates in AL amyloidosis. OBJECTIVES: This study 
      sought to evaluate the effect of BDex+AA as a first-line treatment strategy on 
      mortality in patients with symptomatic heart failure from AL cardiac amyloidosis. 
      METHODS: Patients newly diagnosed with symptomatic New York Heart Association 
      (NYHA) functional class >/=II heart failure due to AL amyloidosis were 
      retrospectively studied. Initial treatment strategy was adjudicated and 
      propensity score analysis was used to adjust for the nonrandomized allocation of 
      treatments. Survival was assessed using a Cox proportional hazards model after 
      adjusting for the propensity score for receiving treatment, age, NYHA functional 
      class, and ejection fraction. RESULTS: Among 106 treated patients (age 64.6 +/- 
      11.3 years, 63% male, 76% lambda subtype), 40 received the 3-drug regimen and 66 
      received other regimens. Mortality was 65% overall, 48% in the BDex+AA cohort 
      (median survival time 821 days), and 76% in patients who received other regimens 
      (median survival time 223 days). Initial treatment with BDex+AA was associated 
      with decreased mortality after multivariable adjustment (hazard ratio: 0.209; 95% 
      confidence interval: 0.069 to 0.636; p = 0.006). This association remained after 
      further adjustment for components of the Mayo Stage. CONCLUSIONS: Use of BDex+AA 
      in the treatment of AL amyloidosis in patients presenting with symptomatic heart 
      failure is associated with improved survival after adjusting for clinical 
      variables.
CI  - Copyright (c) 2016 American College of Cardiology Foundation. Published by Elsevier 
      Inc. All rights reserved.
FAU - Sperry, Brett W
AU  - Sperry BW
AD  - Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, 
      Ohio. Electronic address: sperryb@ccf.org.
FAU - Ikram, Asad
AU  - Ikram A
AD  - Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, 
      Ohio.
FAU - Hachamovitch, Rory
AU  - Hachamovitch R
AD  - Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, 
      Ohio.
FAU - Valent, Jason
AU  - Valent J
AD  - Department of Hematology, Cleveland Clinic Foundation, Cleveland, Ohio.
FAU - Vranian, Michael N
AU  - Vranian MN
AD  - Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, 
      Ohio.
FAU - Phelan, Dermot
AU  - Phelan D
AD  - Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, 
      Ohio.
FAU - Hanna, Mazen
AU  - Hanna M
AD  - Department of Cardiovascular Medicine, Cleveland Clinic Foundation, Cleveland, 
      Ohio.
LA  - eng
PT  - Journal Article
PL  - United States
TA  - J Am Coll Cardiol
JT  - Journal of the American College of Cardiology
JID - 8301365
RN  - 0 (Alkylating Agents)
RN  - 69G8BD63PP (Bortezomib)
RN  - 7S5I7G3JQL (Dexamethasone)
SB  - IM
CIN - J Am Coll Cardiol. 2016 Jun 28;67(25):2949-51. PMID: 27339492
MH  - Aged
MH  - Alkylating Agents/*therapeutic use
MH  - Amyloidosis/*complications/*drug therapy
MH  - Bortezomib/*therapeutic use
MH  - Dexamethasone/*therapeutic use
MH  - Drug Therapy, Combination
MH  - Female
MH  - Heart Diseases/*complications/*drug therapy
MH  - Heart Failure/diagnosis/*etiology/*mortality
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Retrospective Studies
MH  - Survival Rate
MH  - Treatment Outcome
OTO - NOTNLM
OT  - bortezomib
OT  - congestive heart failure
OT  - cyclophosphamide
OT  - infiltrative cardiomyopathy
OT  - light chain
OT  - melphalan
EDAT- 2016/06/25 06:00
MHDA- 2017/06/27 06:00
CRDT- 2016/06/25 06:00
PHST- 2016/02/23 00:00 [received]
PHST- 2016/03/25 00:00 [revised]
PHST- 2016/03/29 00:00 [accepted]
PHST- 2016/06/25 06:00 [entrez]
PHST- 2016/06/25 06:00 [pubmed]
PHST- 2017/06/27 06:00 [medline]
AID - S0735-1097(16)32928-X [pii]
AID - 10.1016/j.jacc.2016.03.593 [doi]
PST - ppublish
SO  - J Am Coll Cardiol. 2016 Jun 28;67(25):2941-8. doi: 10.1016/j.jacc.2016.03.593.