Fontan: The Australian and New Zealand (ANZ) Fontan Registry
The Australian and New Zealand Fontan Registry is a population-based registry collecting health information on all patients who have undergone the Fontan procedure (used to treat single-ventricle congenital heart disease) living in Australia and New Zealand. Recruitment is ongoing and, as of the end of 2017, there are 1,574 registered patients. It is the largest Fontan database in the world, providing researchers and doctors with information to help improve treatment options and long-term health outcomes for patients.
The Fontan procedure has only been performed for 40 years, and there are still many things researchers do not know about its long-term impact and effectiveness. Some patients have seen their condition deteriorate over time, with some requiring heart transplantation. The reasons for this deterioration are not yet clear and researchers do not know how many patients will be affected and when.
The aims of the Registry are to facilitate understanding of the changing composition and health outcomes of this cohort through population-based, longitudinal follow up; better delineate associations between short-term and long-term outcomes and modifiable factors; provide vital information for health service provision and planning; and enable recruitment for prospective, randomised trials.
Participants of the Registry have participated in a number of cross-sectional/one-off sub-studies. A brief description of the major sub-studies can be found in the sub-studies tab below.
|Year||2009 – ongoing (but includes all individuals in Australia and New Zealand who have undergone the Fontan surgical procedure)|
|Age||18.1 (1.0 – 52.5) years|
|#||1,574 (as of the end of 2017)|
|Time point||Enrolment||Pre-surgery||Surgery||Follow-up visit(s)||Follow-up event(s)|
|Condition specific information|
|General health||General health|
|Medications and supplements|
|Physical activity and lifestyle|
|Reproductive health and pregnancy|
|Linkage to other database(s)|
The Australian and New Zealand Fontan Registry has a number of cross-sectional or one-off studies currently underway or recently completed. Please see below for a brief list and description of a selection of studies. For more information about a particular study, please contact the Registry here.
- Screening of Fontan patients in Australia and New Zealand for long-term liver and kidney damage (NHMRC funded) (2013-2015) (N = 152)
- Functional outcomes after Fontan surgery examining anti-coagulation and fenestration in relation to bone density, cardiac, exercise and neurologic outcomes (NHMRC-funded) (2014-2017)
- Neurocognitive outcomes in children and adults with a Fontan circulation (HeartKids funded) (2016-2017)
- Transition from paediatric to adult care for individuals with a Fontan circulation (NHMRC funded) (2014-2019)
- From the Heart study examining wellbeing and quality of life in individuals with a Fontan circulation and their families (NHMRC funded) (2014-2019)
- Pregnancy outcomes in women with a Fontan circulation (ongoing)
|Study name||The Australian and New Zealand (ANZ) Fontan Registry|
|Current principal investigator/s||Prof Yves d’Udekem|
|Primary Institution||Murdoch Children’s Research Institute|
|Collaborating Institution/s||All paediatric and adult congenital heart disease centres across Australia and New Zealand|
|Major funding sources||National Health and Medical Research Council
|Key reference for study (published study protocol)||
Iyengar, A.J., Winlaw, D.S., Galati, J.C., Gentles, T.L., Weintraub, R.G., Justo, R.N., Wheaton, G.R., Bullock, A., Celermajer, D.S. and d’Udekem, Y. (2014). The Australia and New Zealand Fontan Registry: description and initial results from the first population-based Fontan registry. Intern Med J, 44(2): 148-55. doi: 10.1111/imj.12318
d’Udekem, Y., Iyengar, A.J., Galati, J.C., Forsdick, V., Weintraub, R.G., Wheaton, G.R., Bullock, A., Justo, R.N., Grigg, L.E., Sholler, G.F., Hope, S., Radford, D.J., Gentles, T.L., Celermajer, D.S. and Winlaw, D.S. (2014). Redefining expectations of long-term survival after the Fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand. Circulation, 130(11 Suppl 1): S32-8. doi: 10.1161/circulationaha.113.007764
|Are data available to others outside study team?||On request|
|Study focus (e.g. social development)||Health and wellbeing outcomes post-Fontan surgery.|
|Sampling frame||All individuals in Australia and New Zealand who have undergone the Fontan surgical procedure since it was first described in 1971.|
|Study type (e.g. randomised control trial, cohort, case-control)||Register|
|Year commenced||The Fontan Registry commenced in 2009, but all individuals who have had the Fontan surgical procedure in Australia and New Zealand are included.|
|Current sample (N)||1,574 at the end of 2017|
|Annual recruitment rate||Approximately 60 new cases per year|
|Intergenerational (e.g. offspring)?||No|
|Imaging (e.g. fMRI, ultrasound, retinal photograph)?||Echocardiogram
|Linkage (e.g. BioGrid, VPDC, NAPLAN, Medicare)?||National Death Index (NDI)
Medicare Benefits Scheme (MBS)/Pharmaceutical Benefits Scheme (PBS) (consent currently underway)
|Biosamples (e.g. buccal, blood, hair)?||No|
|Ethics approvals or requirements (e.g. specific, extended, unspecified, other)?||Opt-out consent for registry.
Associated studies have individual consent.
|Year||Age (mean, range)||Eligible sample (not deceased, not withdrawn)|
|Procedure years: 1971 – ongoing||18.1 (1.0 – 52.5) years||1,574 as of the end of 2017|
Prof Yves d’Udekem
Ph: +61 9345 5200
Murdoch Childrens Research Institute
The Royal Children’s Hospital
50 Flemington Road