Fontan for Life

William was just 38 hours old when he underwent his first heart surgery.  In his short life he has undergone three heart surgeries at the Heart Centre for Children – culminating in the Fontan procedure which he had last year. Now, at six years of age, his mother Lisa Chamberlin says he is a completely different child, who is active and loves to run around with his brother. William is one of over 900 children and adults who are taking part in the largest heart study of its kind in the world, the Fontan Registry and who, this month is hoping to raise awareness of the registry during a campaign called ‘Fontan for Life’ which aims to raise $200,000 for the registry.

The Fontan procedure is a lifesaving medical technique used to treat children born with certain serious heart defects. The procedure involves restructuring the heart so that it can operate effectively using only one ventricle, or pumping chamber, as opposed to two.

The Fontan procedure is relatively new in medical terms, and has only been around for 40 years.  The registry was established two years ago so researchers and surgeons could follow up patients living in Australia and New Zealand and improve their health outcomes and quality of life, and at the Heart Centre for Children we are heavily involved in keeping the registry going.

Already the registry is seeing results; new research has found that doomsayers, who said the Fontan operation would last less than 20 years, have been proven wrong.  In the largest follow up study of its kind, and the only one based on entire populations, researchers found the best possible results in terms of survival for this group of heart patients. 

“We are now proving that the vast majority of these patients will survive for three decades,” said Murdoch Childrens A/Professor and heart surgeon Yves d’Udekem, “it is therefore likely a lot of these patients will see their fourth and even fifth decade post surgery.”  

Further research from the registry has found that the Fontan population is increasing rapidly.  Whilst this used to be a rare procedure, the population will continue to grow and in 30 years will grow to over 3,000 patients in Australia - which is the same number of kids with cystic fibrosis- a disease everyone has heard of.

“The registry is already improving the care of these patients,” Yves said, “However, I want to be able to tell my patients what kind of life they can expect and what effect this procedure will have on their heart, lungs, kidneys, liver and even their brain.” 

William’s mother Lisa says the Fontan has already had a profound impact on William’s life.

“I can’t speak more highly of the impact it has had.  The Fontan has had a huge impact on William’s quality of life.  Before the procedure William was active but he would become breathless and tire a lot quicker.  Now it’s like he is a different child, he is so much healthier,” Lisa said. 

“I am really happy William is involved in the registry as I really want to know more about the long term health outcomes and anything that could potentially prolong his life.” 

Pilot data from the registry has found one in three patients stop seeing their specialist cardiologist once they become adults. This is a huge problem, because potential problems get missed until it’s too late to do anything. A pilot study that is screening patients for liver and kidney failure has found problems in half the patients, mostly with no symptoms. 

The money raised from the ‘Fontan for Life’ campaign will help fund a number of crucial projects, including this study that will investigate the liver and kidney function of patients who have undergone the Fontan procedure.   The project is important in determining how these two major organs tolerate the higher vein pressures generated after Fontan surgery and findings from this research will ultimately improve the care that Fontan patients receive.

Funds raised will also support a fertility and childbearing study.  It is recognised that pregnancy is high risk for women with a Fontan. The study aims to collect data regarding fertility, pregnancy advice and complications, as well as delivery and outcomes for Fontan mothers and their babies.

In the future the registry will also be utilised to initiate large scale trials for potential new medications and treatments.

“With the population of Fontan recipients growing each year, there is a need to develop longer-term solutions to offer these patients a greater life expectancy. Ultimately, we hope this registry will help identify the needs of these patients now and in the future, so that measures can be taken to ensure they receive the best possible care and life expectancy.”

“We’re also creating an online community to help patients unite and share information with each other and to help guide us to do research projects that are relevant to them.”

The collaborative work is the first of its kind in the world, with all cardiac specialists in Australia and New Zealand working jointly on the project. 

To donate visit www.fontanforlife.com.au or to find out more about the registry visit www.fontanregistry.com.au.