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

BackgroundThere 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.ObjectiveWe decided to review the literature to better establish the efficacy of ACE inhibitors in the single ventricle population and summarise their indications.MethodsA 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.ResultsA search in the Ovid MEDLINE and Embase databases identified a total of 140 articles, of which 31 were deemed relevant to the review.ConclusionsThere 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.