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 July 2017, there are 1,459 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 19 (2.9-67.5) years
# 1,459 (as of July 2017)
Time point Enrolment Pre-surgery Surgery Follow-up visit(s) Follow-up event(s)
Anthropometrics
Birth Birth
Cardiovascular health
Condition specific information
Demographics
General health General health
Imaging Imaging
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 Summary
Study name The Australian and New Zealand (ANZ) Fontan Registry
Current principal investigator/s Prof Yves d’Udekem
Current project manager Dr Karin du Plessis
Primary Institution Murdoch Childrens 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
Heart Foundation
HeartKids
Study website www.fontanregistry.com
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.
Ongoing recruitment? Yes
Current sample (N) 1,459 as of July 2017
Annual recruitment rate Approximately 60 new cases per year
Intergenerational (e.g. offspring)? No
Imaging (e.g. fMRI, ultrasound, retinal photograph)? Echocardiogram
MRI
Cath lab
Linkage (e.g. BioGrid, VPDC, NAPLAN, Medicare)? National Death Index (NDI)
Medicare Benefits Scheme (MBS)/Pharmaceutical Benefits Scheme (PBS) (consent currently underway)
Medical records
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 19 (2.9-67.5) years 1,459 as of July 2017

Principal Investigator

Prof Yves d’Udekem

Project Manager

Dr Karin du Plessis

Study Contact

Ph: +613 9345 6161
Email: info@fontanregistry.com

Postal Address

Fontan Registry
Murdoch Childrens Research Institute
The Royal Children’s Hospital
50 Flemington Road
Parkville
VIC 3052