The Fontan epidemic: Population projections from the Australia and New Zealand Fontan Registry

Chris Schilling; Kim Dalziel; Russell Nunn; Karin Du Plessis; William Y Shi; David Celermajer; David Winlaw; Robert G Weintraub; Leanne E Grigg; Dorothy J Radford; Andrew Bullock; Thomas L Gentles; Gavin R Wheaton; Tim Hornung; Robert N Justo; Yves d'Udekem

doi:10.1016/j.ijcard.2016.05.035

The Fontan epidemic: Population projections from the Australia and New Zealand Fontan Registry

Int J Cardiol. 2016 Sep 15:219:14-9. doi: 10.1016/j.ijcard.2016.05.035. Epub 2016 May 14.

Authors

Chris Schilling¹, Kim Dalziel¹, Russell Nunn², Karin Du Plessis³, William Y Shi⁴, David Celermajer⁵, David Winlaw⁶, Robert G Weintraub⁷, Leanne E Grigg⁸, Dorothy J Radford⁹, Andrew Bullock¹⁰, Thomas L Gentles¹¹, Gavin R Wheaton¹², Tim Hornung¹¹, Robert N Justo¹³, Yves d'Udekem¹⁴

Affiliations

¹ Centre for Health Policy, The University of Melbourne, 207 Bouverie St Carlton, Victoria 3051, Australia.
² Department of Cardiac Surgery, Royal Children's Hospital, Flemington Rd, Parkville, Victoria 3052, Australia.
³ Murdoch Childrens Research Institute, Melbourne, Victoria, Australia.
⁴ Murdoch Childrens Research Institute, Melbourne, Victoria, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Victoria, Australia.
⁵ Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia; Department of Medicine, The University of Sydney, New South Wales, Australia.
⁶ Heart Centre for Children, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.
⁷ Department of Cardiology, Royal Children's Hospital, Melbourne, Victoria, Australia.
⁸ Department of Cardiology, The Royal Melbourne Hospital, Melbourne, Victoria, Australia.
⁹ Adult Congenital Heart Unit, The Prince Charles Hospital, Brisbane, Queensland, Australia; Department of Medicine, University of Queensland, Brisbane, Australia.
¹⁰ Children's Cardiac Centre, Princess Margaret Hospital for Children, Perth, Western Australia, Australia.
¹¹ Green Lane Paediatric and Congenital Cardiac Service, Starship Children's Hospital Auckland, Auckland, New Zealand.
¹² Department of Cardiology, Women's and Children's Hospital, Adelaide, South Australia, Australia.
¹³ Queensland Paediatric Cardiac Service, Lady Cilento Children's Hospital, Queensland, Australia.
¹⁴ Department of Cardiac Surgery, Royal Children's Hospital, Flemington Rd, Parkville, Victoria 3052, Australia; Murdoch Childrens Research Institute, Melbourne, Victoria, Australia; Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Melbourne, Victoria, Australia. Electronic address: yves.dudekem@rch.org.au.

PMID: 27257850
DOI: 10.1016/j.ijcard.2016.05.035

Abstract

Background: The number and age demographic of the future Fontan population is unknown.

Methods: Population projections were calculated probabilistically using microsimulation. Mortality hazard rates for each Fontan recipient were calculated from survivorship of 1353 Fontan recipients in the Australia and New Zealand Fontan Registry, based on Fontan type, age at Fontan, gender and morphology. Projected rates of new Fontan procedures were generated from historical rates of Fontan procedures per population births.

Results: At the end of 2014, the living Fontan population of Australia and New Zealand was 1265 people from an Australian and New Zealand regional population of 28 million (4.5 per 100,000 population). Of those, 165 (13%) received an atrio-pulmonary (AP) procedure, 262 (21%) a lateral tunnel (LT) procedure and 838 (66%) an extra-cardiac conduit (ECC) procedure. This population is expected to grow to 1917 (95% CI: 1846: 1986) by 2025 (5.8 per 100,000 population), with 149 (8%) AP procedures, 254 (13%) LT procedures, and 1514 (79%) ECC procedures. By 2045, the living Fontan population is expected to reach 2986 (95% CI: 2877: 3085; 7.2 per 100,000 population). The average age of the Fontan population is expected to increase from 18years in 2014 to 23years (95% CI: 22-23) by 2025, and 31years (95% CI: 30-31) by 2045.

Conclusion: The Australian and New Zealand population of patients alive after a Fontan procedure will double over the next 20years increasing the demand for heart-failure services and cardiac transplantation. Greater consideration for the needs of this mostly adult Fontan population will be necessary.

Keywords: Congenital heart disease; Fontan procedure; Modelling; Survival.

MeSH terms

Adolescent
Adult
Australia / epidemiology
Child
Female
Fontan Procedure / mortality*
Fontan Procedure / trends*
Humans
Male
New Zealand / epidemiology
Population Forecast / methods*
Registries*
Survival Rate / trends
Young Adult