title: Use of ACE inhibitors in Fontan: Rational or irrational?
abstract: BACKGROUND
  Despite a lack of evidence supporting the use of angiotensin-converting enzyme (ACE) inhibitors in patients with a Fontan circulation, their use is frequent. We decided to identify the rationale for ACE inhibitor therapy in patients within the Australia and New Zealand Fontan Registry.
  
  
  METHODS
  All patients in the Registry taking an ACE inhibitor at last follow up were identified, and a review of medical records was undertaken to determine the rationale for treatment initiation and reasons for treatment continuation or dose increase.
  
  
  RESULTS
  In 2015, 36% of the surviving patients in the Registry (462/1268) were taking an ACE inhibitor. Indications for initiation of therapy were ventricular systolic or diastolic dysfunction (29%), atrioventricular valve regurgitation (19%), preservation of normal ventricular function (7%), prolonged effusions at Fontan (6%), hypertension (6%), other (6%) and unknown (2%). No indication was stated in the remaining patients (25%). Those with hypoplastic left heart syndrome were more likely to be on an ACE inhibitor than those with an alternative primary morphology (70% vs 32%; p<0.001). Only 36% of the patients treated with an ACE inhibitor at last follow up (166/462) had an indication that would generally justify treatment in a two-ventricle circulation.
  
  
  CONCLUSIONS
  It is likely that the use of ACE inhibitors in patients with a Fontan circulation is excessive within our region. The coordination of prospective, multicentre studies and initiatives such as the Australia and New Zealand Fontan Registry will facilitate further investigations to guide treatment decisions in the growing Fontan population.
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Heart Research Group, Murdoch Childrens Research Institute, Melbourne, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Victoria, Australia.
authors.forename: Thomas G
authors.initials: TG
authors.lastname: Wilson
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Heart Research Group, Murdoch Childrens Research Institute, Melbourne, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Victoria, Australia.
authors.forename: Ajay J
authors.initials: AJ
authors.lastname: Iyengar
authors.affiliation: The Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia; Department of Paediatrics, University of Sydney, Sydney, Australia.
authors.forename: David S
authors.initials: DS
authors.lastname: Winlaw
authors.affiliation: Heart Research Group, Murdoch Childrens Research Institute, Melbourne, Australia; Department of Cardiology, Royal Children's Hospital, Melbourne, Australia.
authors.forename: Robert G
authors.initials: RG
authors.lastname: Weintraub
authors.affiliation: Department of Cardiology, Women's and Children's Hospital, Adelaide, Australia.
authors.forename: Gavin R
authors.initials: GR
authors.lastname: Wheaton
authors.affiliation: Greenlane Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand.
authors.forename: Thomas L
authors.initials: TL
authors.lastname: Gentles
authors.affiliation: The Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia; Department of Paediatrics, University of Sydney, Sydney, Australia.
authors.forename: Julian
authors.initials: J
authors.lastname: Ayer
authors.affiliation: Department of Cardiology, The Royal Melbourne Hospital, Melbourne, Australia.
authors.forename: Leeanne E
authors.initials: LE
authors.lastname: Grigg
authors.affiliation: Queensland Paediatric Cardiac Service, Lady Cilento Children's Hospital, Brisbane, Queensland, Australia.
authors.forename: Robert N
authors.initials: RN
authors.lastname: Justo
authors.affiliation: Adult Congenital Heart Unit, The Prince Charles Hospital, Brisbane, Australia; Faculty of Medicine, University of Queensland, Brisbane, Victoria, Australia.
authors.forename: Dorothy J
authors.initials: DJ
authors.lastname: Radford
authors.affiliation: Children's Cardiac Centre, Princess Margaret Hospital for Children, Perth, Australia.
authors.forename: Andrew
authors.initials: A
authors.lastname: Bullock
authors.affiliation: Department of Paediatrics, University of Sydney, Sydney, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
authors.forename: David S
authors.initials: DS
authors.lastname: Celermajer
authors.affiliation: Melbourne School of Population and Global Health, The University of Melbourne, Victoria, Australia.
authors.forename: Kim
authors.initials: K
authors.lastname: Dalziel
authors.affiliation: Melbourne School of Population and Global Health, The University of Melbourne, Victoria, Australia.
authors.forename: Chris
authors.initials: C
authors.lastname: Schilling
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Heart Research Group, Murdoch Childrens Research Institute, Melbourne, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Victoria, Australia. Electronic address: yves.dudekem@rch.org.au.
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
mesh_terms: D000293:Adolescent; D000328:Adult; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D001315:Australia / Q000453:epidemiology; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000188:drug therapy* / Q000453:epidemiology / Q000601:surgery*; D006801:Humans; D008297:Male; D009520:New Zealand / Q000453:epidemiology; D012042:Registries; D055815:Young Adult
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors
keywords: Angiotensin-converting enzyme inhibitors; Fontan procedure; Heart defects congenital; Heart single ventricle; Heart ventricles abnormalities