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Fontan ACE inhibitor randomised controlled trial for nephropathy

Dr Rachael Cordina, Institution: Murdoch Children's Research Institute

2017 Vanguard Grant

Years funded: 2018-2022


The Fontan procedure is the last in a series of operations offered to children born with hearts with a single ventricle, or pumping chamber.

These patients are now surviving into adulthood, however, are susceptible to multiple complications including arrhythmias, thromboembolic events, heart failure, and liver and kidney problems.

Utilising the Australian and New Zealand Fontan Registry, a database comprising over 1400 Fontan recipients, we have recently conducted a NHMRC-funded cross-sectional study investigating liver and kidney disease in these patients.

Although currently unpublished, this data has revealed that one-third of Fontan patients have evidence of nephropathy (kidney disease or damage), and one-third have mild or moderate impairment in kidney function.

It was also discovered that the degree of nephropathy increases with time since Fontan completion. ACE inhibitors are a class of drugs that are effective in improving kidney function and preventing progression of nephropathy in a number of settings, however their benefit in Fontan patients are uncertain.

The aim of this study is to conduct a trial to test the efficacy of ACE inhibitors in Fontan patients with established nephropathy. Demonstrating a clinical benefit of ACE inhibitors in Fontan patients would result in a paradigm shift in the management of these patients and contribute towards prevention of end-organ dysfunction in this vulnerable population.

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