PMID- 30686641
OWN - NLM
STAT- MEDLINE
DCOM- 20200603
LR  - 20211204
IS  - 1444-2892 (Electronic)
IS  - 1443-9506 (Linking)
VI  - 29
IP  - 1
DP  - 2020 Jan
TI  - Sacubitril/Valsartan in Adult Congenital Heart Disease Patients With Chronic 
      Heart Failure - A Single Centre Case Series and Call for an International 
      Registry.
PG  - 137-141
LID - S1443-9506(18)32031-6 [pii]
LID - 10.1016/j.hlc.2018.12.003 [doi]
AB  - BACKGROUND: An improvement in life expectancy in patients suffering from adult 
      congenital heart disease (ACHD) has corresponded with a rise in heart failure 
      incidence within this group. An area that has not been addressed in ACHD heart 
      failure guidelines has been the use of combined inhibition of angiotensin 
      receptor-neprolysin pathways. This case series sought to demonstrate tolerability 
      and 6-month outcome measures of sacubitril/valsartan use in ACHD patients with a 
      severely impaired systemic ventricle. METHODS: A prospective cohort analysis of 
      ACHD patients between December 2016 and September 2017 with severe systemic 
      ventricular systolic dysfunction, New York Heart Association (NYHA) class II-III 
      symptoms and eligible for commencement on a sacubitril/valsartan regime was 
      undertaken. RESULTS: Five (5) consecutive patients were included in this cohort 
      review, 80% male, mean age 41.8 (+/-19) years and mean systemic ventricular 
      ejection fraction 27% (+/-3.6%). Two (2) patients with pre-existing D-TGA and 
      atrial baffle repair, one patient with Tetralogy of Fallot repair and pulmonary 
      valve replacement (PVR), one patient with left atrial isomerism and partial 
      atrioventricular (AV) canal defect repair and mitral valve replacement (MVR) and 
      the last patient had biventricular repair for pulmonary atresia with MVR and PVR. 
      Forty per cent (40%) of patients had a systemic right ventricle. All patients had 
      NYHA functional class of >/=II, were on optimal tolerated doses ACE-I or ARB prior 
      to sacubitril/valsartan combination. Six (6) months post commencement of 
      sacubitril/valsartan patients experienced a mean improvement of one functional 
      class. CONCLUSIONS: Our experience suggests that sacubitril/valsartan therapy is 
      well tolerated in ACHD heart failure patients and is associated with improvement 
      in functional class.
CI  - Crown Copyright (c) 2018. Published by Elsevier B.V. All rights reserved.
FAU - Appadurai, Vinesh
AU  - Appadurai V
AD  - Department of Cardiology, Mater Hospital Services, Brisbane, Qld, Australia; 
      Department of Cardiology, Princess Alexandra Hospital, Metro South Hospital and 
      Health Services, Brisbane, Qld, Australia; School of Medicine, The University of 
      Queensland, Brisbane, Qld, Australia. Electronic address: 
      vinesh.appadurai@health.qld.gov.au.
FAU - Thoreau, Jennifer
AU  - Thoreau J
AD  - Department of Cardiology, Mater Hospital Services, Brisbane, Qld, Australia; 
      Department of Cardiology, Princess Alexandra Hospital, Metro South Hospital and 
      Health Services, Brisbane, Qld, Australia.
FAU - Malpas, Theresa
AU  - Malpas T
AD  - Department of Cardiology, The Prince Charles Hospital, Metro North Hospital and 
      Health Service, Brisbane, Qld, Australia.
FAU - Nicolae, Mugur
AU  - Nicolae M
AD  - Department of Cardiology, Mater Hospital Services, Brisbane, Qld, Australia; 
      School of Medicine, The University of Queensland, Brisbane, Qld, Australia; 
      Department of Cardiology, The Prince Charles Hospital, Metro North Hospital and 
      Health Service, Brisbane, Qld, Australia.
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20181217
PL  - Australia
TA  - Heart Lung Circ
JT  - Heart, lung & circulation
JID - 100963739
RN  - 0 (Aminobutyrates)
RN  - 0 (Biphenyl Compounds)
RN  - 0 (Drug Combinations)
RN  - 0 (Tetrazoles)
RN  - 80M03YXJ7I (Valsartan)
RN  - WB8FT61183 (sacubitril and valsartan sodium hydrate drug combination)
SB  - IM
MH  - Adult
MH  - Aminobutyrates/*administration & dosage
MH  - Biphenyl Compounds
MH  - Chronic Disease
MH  - Drug Combinations
MH  - Female
MH  - Heart Defects, Congenital/physiopathology/*therapy
MH  - Heart Failure/physiopathology/*therapy
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Prospective Studies
MH  - *Registries
MH  - Tetrazoles/*administration & dosage
MH  - Valsartan/*administration & dosage
OTO - NOTNLM
OT  - Adult congenital heart disease
OT  - Congestive cardiac failure
OT  - Sacubitril-Valsartan
EDAT- 2019/01/29 06:00
MHDA- 2020/06/04 06:00
CRDT- 2019/01/29 06:00
PHST- 2018/07/26 00:00 [received]
PHST- 2018/11/04 00:00 [revised]
PHST- 2018/12/06 00:00 [accepted]
PHST- 2019/01/29 06:00 [pubmed]
PHST- 2020/06/04 06:00 [medline]
PHST- 2019/01/29 06:00 [entrez]
AID - S1443-9506(18)32031-6 [pii]
AID - 10.1016/j.hlc.2018.12.003 [doi]
PST - ppublish
SO  - Heart Lung Circ. 2020 Jan;29(1):137-141. doi: 10.1016/j.hlc.2018.12.003. Epub 
      2018 Dec 17.