The Use and Misuse of ACE Inhibitors in Patients with Single Ventricle Physiology

Heart Lung Circ. 2016 Mar;25(3):229-36. doi: 10.1016/j.hlc.2015.10.005. Epub 2015 Oct 27.


Background: There are no clear guidelines for the use of angiotensin-converting enzyme (ACE) inhibitors in patients with single ventricle physiology, especially after the Fontan procedure. Despite this, ACE inhibitors are widely prescribed within the single ventricle population.

Objective: We decided to review the literature to better establish the efficacy of ACE inhibitors in the single ventricle population and summarise their indications.

Methods: A review of the literature was conducted to identify relevant articles describing use of ACE inhibitors in single ventricle patients both before and after the Fontan procedure.

Results: A search in the Ovid MEDLINE and Embase databases identified a total of 140 articles, of which 31 were deemed relevant to the review.

Conclusions: There is little published evidence supporting the use of ACE inhibitors in patients with single ventricle physiology. Based on the current literature, it is possible that ACE inhibitors are overprescribed in patients with single ventricle physiology. The coordination of large, prospective studies through initiatives such as the Australia and New Zealand Fontan Registry is necessary to guide the appropriate use of ACE inhibitors in the single ventricle population.

Keywords: Angiotensin-converting enzyme inhibitors; Fontan procedure; Heart defects congenital; Heart single ventricle; Heart ventricles abnormalities.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Angiotensin-Converting Enzyme Inhibitors / adverse effects*
  • Angiotensin-Converting Enzyme Inhibitors / therapeutic use*
  • Female
  • Heart Diseases* / drug therapy
  • Heart Diseases* / physiopathology
  • Heart Ventricles* / abnormalities
  • Heart Ventricles* / physiopathology
  • Humans
  • Male


  • Angiotensin-Converting Enzyme Inhibitors