PMID- 30660664
OWN - NLM
STAT- MEDLINE
DCOM- 20191206
LR  - 20191217
IS  - 1532-8414 (Electronic)
IS  - 1071-9164 (Linking)
VI  - 25
IP  - 3
DP  - 2019 Mar
TI  - Clinical Experience With the Use of Doxycycline and Ursodeoxycholic Acid for the 
      Treatment of Transthyretin Cardiac Amyloidosis.
PG  - 147-153
LID - S1071-9164(18)30927-8 [pii]
LID - 10.1016/j.cardfail.2019.01.006 [doi]
AB  - BACKGROUND: The tolerability and utility of combination doxycycline and 
      ursodeoxycholic acid (ursodiol) amyloid fibril disruption therapy for 
      transthyretin cardiac amyloidosis (ATTR CA) in clinical practice is poorly 
      described. METHODS AND RESULTS: We report the clinical experience of 53 ATTR CA 
      patients treated with doxycycline and ursodiol. Six patients (11%) did not 
      tolerate the therapy owing to dermatologic and gastrointestinal effects. Of those 
      remaining, the median follow-up was 22 months (range 8-30), mean age was 71 +/- 
      11years, 41 (87%) were male, and 42 (89%) had wild-type and 5 (11%) mutant ATTR. 
      Five patients (11%) died during follow-up. There was no significant change in New 
      York Heart Association (NYHA) functional class, cardiac biomarkers, or 
      echocardiographic parameters during follow-up. Left ventricular (LV) global 
      longitudinal systolic strain (GLS) improved in 16 patients (38%) (-12 +/- 4% to -17 
      +/- 4%; P < .01). Patients whose LV GLS improved were significantly younger and had 
      lower NYHA functional class, troponin-T, N-terminal pro-B-type natriuretic 
      peptide (BNP), and baseline LV GLS levels compared with those whose LV GLS did 
      not improve. Troponin-T improved in follow-up for patients whose LV GLS improved 
      (35 +/- 21 to 20 +/- 14 ng/L; P = .06). CONCLUSIONS: Doxycycline and ursodiol therapy 
      for treatment of ATTR CA was tolerable and was associated with stabilized markers 
      of disease progression. LV GLS improved in patients with less advanced disease.
CI  - Copyright (c) 2019 Elsevier Inc. All rights reserved.
FAU - Karlstedt, Erin
AU  - Karlstedt E
AD  - Division of Cardiology, Department of Cardiac Sciences, Libin Cardiovascular 
      Institute of Alberta, Calgary, Alberta, Canada.
FAU - Jimenez-Zepeda, Victor
AU  - Jimenez-Zepeda V
AD  - Division of Hematology, Department of Medicine, Cumming School of Medicine, 
      University of Calgary, Calgary, Alberta, Canada.
FAU - Howlett, Jonathan G
AU  - Howlett JG
AD  - Division of Cardiology, Department of Cardiac Sciences, Libin Cardiovascular 
      Institute of Alberta, Calgary, Alberta, Canada.
FAU - White, James A
AU  - White JA
AD  - Division of Cardiology, Department of Cardiac Sciences, Libin Cardiovascular 
      Institute of Alberta, Calgary, Alberta, Canada.
FAU - Fine, Nowell M
AU  - Fine NM
AD  - Division of Cardiology, Department of Cardiac Sciences, Libin Cardiovascular 
      Institute of Alberta, Calgary, Alberta, Canada. Electronic address: 
      nmfine@ucalgary.ca.
LA  - eng
PT  - Journal Article
PT  - Observational Study
DEP - 20190118
PL  - United States
TA  - J Card Fail
JT  - Journal of cardiac failure
JID - 9442138
RN  - 0 (Amyloid)
RN  - 0 (Anti-Bacterial Agents)
RN  - 0 (Cholagogues and Choleretics)
RN  - 0 (Prealbumin)
RN  - 0 (TTR protein, human)
RN  - 724L30Y2QR (Ursodeoxycholic Acid)
RN  - N12000U13O (Doxycycline)
SB  - IM
CIN - J Card Fail. 2019 Mar;25(3):154-155. PMID: 30726714
MH  - Aged
MH  - Aged, 80 and over
MH  - Amyloid/antagonists & inhibitors/metabolism
MH  - Amyloidosis/*drug therapy/metabolism
MH  - Anti-Bacterial Agents/administration & dosage
MH  - Cholagogues and Choleretics/administration & dosage
MH  - Cohort Studies
MH  - Doxycycline/*administration & dosage
MH  - Drug Therapy, Combination
MH  - Female
MH  - Follow-Up Studies
MH  - Heart Diseases/*drug therapy/metabolism
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - *Prealbumin/metabolism
MH  - Treatment Outcome
MH  - Ursodeoxycholic Acid/*administration & dosage
OTO - NOTNLM
OT  - Transthyretin cardiac amyloidosis
OT  - doxycycline
OT  - efficacy
OT  - tolerability
OT  - ursodiol
EDAT- 2019/01/21 06:00
MHDA- 2019/12/18 06:00
CRDT- 2019/01/21 06:00
PHST- 2018/08/21 00:00 [received]
PHST- 2019/01/07 00:00 [revised]
PHST- 2019/01/15 00:00 [accepted]
PHST- 2019/01/21 06:00 [pubmed]
PHST- 2019/12/18 06:00 [medline]
PHST- 2019/01/21 06:00 [entrez]
AID - S1071-9164(18)30927-8 [pii]
AID - 10.1016/j.cardfail.2019.01.006 [doi]
PST - ppublish
SO  - J Card Fail. 2019 Mar;25(3):147-153. doi: 10.1016/j.cardfail.2019.01.006. Epub 
      2019 Jan 18.