The Australia and New Zealand Fontan Registry: the ideal model to improve the adult life of children born with congenital heart disease

Years funded:
2014 - 2019

People born with a single pumping chamber in their heart are at the worst end of the spectrum of congenital heart disease. The Fontan procedure is the last of a series of operations giving them survival to adulthood, but an uncertain future thereafter. We have built the largest and the only population-based database of Fontan patients in the world. There are one thousand people, who have had the procedure, currently living in Australia and this will double within 15 years. Half of the patients in the contemporary cohort suffer complications within 10 years of surgery. Transition to adult care sees a third of these patients dropping from the follow-up that would prevent the development of these complications. With the partnership grant, Associate Professor Yves dUdekem aims to: (1) standardize the follow-up care after Fontan surgery; (2) set up a strategy to improve transition to adult care of all Fontan patients; and (3) study the impact of these strategies on complication rates and costs. The project involves all the adult and paediatric units in Australian hospitals that look after congenital heart disease, as well as HeartKids, a charity who provide support to families of children with heart disease. It is the largest project in this field aimed at improving the survival and the quality of life for those with the most severe congenital heart conditions. The benefits of this initiative will not be restricted to this growing population of Fontan patients: it will undoubtedly improve the care of all people growing up with congenital heart disease.

Researcher Profile

Professor Yves d’Udekem

Institute: Murdoch Childrens Research Institute
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