The Fontan procedure in Australia: A population-based study

Abstract

Since its first description in 1971, the Fontan procedure has become the final stage of palliation for children not considered candidates for a two-ventricle repair. The long-term future of these patients is uncertain, but it is clear that a large number of them will experience failure of their “Fontan circulation.” The proportion of these patients who will eventually require heart transplantation is still unknown and may be altered by the use of emerging medical therapies and by surgical options such as the Fontan conversion. The burden of this new patient population on health systems is difficult to evaluate, but it is reasonable to expect that most of them will require expensive therapies after their third decade with a Fontan circulation. Because the Fontan procedure is offered to such a heterogenous group of patients, it is difficult to evaluate the size of this patient population. The perioperative mortality of this procedure is now minimal, and the number of patients operated on will be the main determinant of the size of the population surviving with a Fontan circulation. In the past 3 decades, the Royal Children’s Hospital has performed all Fontan operations in children from four Australian states; this has allowed us to evaluate the incidence of the Fontan procedure in our Australian population.