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Introduction: The term single ventricle defect (SVD) is used to define a set of congenital cardiac malformations characterised by the failure of correct development of a lower chamber of the heart. Despite the prognosis for these patients being significantly improved by the Fontan procedure, these patients still commonly develop heart failure. Despite being recommended in adult heart failure, the use of angiotensin converting enzyme inhibitors (ACEIs) in paediatric single ventricle physiology is not evidence based. The only randomised controlled trial (RCT) to look into this issue found that ACEIs offer no benefits to these patients and are therefore not recommended. Self-reported ACEI use in single ventricle paediatric patients: Two recent largescale surveys displayed that a significant proportion of the health care community were either not aware of the evidence or had chosen purposefully not to alter their practice. Current best evidence: The only RCT conducted on this issue compared the effects of enalapril and a placebo and found that enalapril conferred no benefits in relation to the chosen endpoints. Future research: The next major study to look into this issue will be phase II the PANORAMA-HF study which will compare entresto (sacubitril/valsartan) with enalapril over a 52 week period. Discussion: The current best evidence is not without flaws and as such it’s validity has been called into question by physicians. Conclusion: Further dissemination of the current research and addition of superior trials to the evidence base is required before ACEI efficacy in this patient group can be truly ascertained.