Pre- and Post-operative determinants of transplantation-free survival after Fontan. The Australia and New Zealand experience

Chin L Poh; Rachael L Cordina; Ajay J Iyengar; Diana Zannino; Leeanne E Grigg; Gavin R Wheaton; Andrew Bullock; Julian Ayer; Nelson Alphonso; Thomas L Gentles; David S Celermajer; Yves d'Udekem

doi:10.1016/j.ijcha.2021.100825

Pre- and Post-operative determinants of transplantation-free survival after Fontan. The Australia and New Zealand experience

Int J Cardiol Heart Vasc. 2021 Jul 7:35:100825. doi: 10.1016/j.ijcha.2021.100825. eCollection 2021 Aug.

Authors

Chin L Poh^{1

2

3}, Rachael L Cordina^{4

5}, Ajay J Iyengar^{1

2

3}, Diana Zannino¹, Leeanne E Grigg⁶, Gavin R Wheaton⁷, Andrew Bullock⁸, Julian Ayer^{9

10}, Nelson Alphonso¹¹, Thomas L Gentles¹², David S Celermajer^{13

14}, Yves d'Udekem^{1

2

3}

Affiliations

¹ Heart Research Group, Murdoch Children's Research Institute, Melbourne, Australia.
² Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Victoria, Australia.
³ Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia.
⁴ Sydney Medical School, University of Sydney, Sydney, Australia.
⁵ Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia.
⁶ Department of Cardiology, The Royal Melbourne Hospital, Melbourne, Vic, Australia.
⁷ Department of Cardiology, Women's and Children's Hospital, Adelaide, South Australia, Australia.
⁸ Department of Cardiology, Perth Children's Hospital, Perth, Western Australia, Australia.
⁹ Heart Centre for Children, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.
¹⁰ Discipline of Paediatrics and Child Health, The University of Sydney, Sydney, New South Wales, Australia.
¹¹ Department of Cardiac Surgery, Queensland Children's Hospital, Brisbane, Australia.
¹² Greenlane Paediatric and Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand.
¹³ Department of Medicine, The University of Sydney, Sydney, New South Wales, Australia.
¹⁴ Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.

Abstract

Background: This review identifies the predictors of late mortality and heart transplantation that remain relevant in the contemporary population of patients with a Fontan circulation, focusing on the potential impact of post-Fontan morbidities on the late outlook of these patients.

Methods and results: A total of 1561 patients who had survived the Fontan operation in Australia or New Zealand from 1975 to 2018 were included in this review. Over a median duration of 11.4 years, there was a total of 117 deaths (7%) and 32 heart transplantations (2%). Freedom from death and heart transplantation at 10, 20 and 35 years post Fontan surgery were 94% (95% CI 93-95%), 87% (95 %CI 85-90%) and 66% (95 %CI 57-78%) respectively. Being male, having an atriopulmonary Fontan, pre-Fontan atrioventricular valve intervention, or prolonged pleural effusions post Fontan were predictive of late death or heart transplantation. However, time-dependent variables such as the development of atrial arrhythmia, protein/losing enteropathy or late ventricular dysfunction were stronger predictors of the same outcome. Patients who developed a time-dependent risk factor had a freedom from death and heart transplantation rate of 54% (95 %CI 43-66) at 15 years and 44% (95 %CI 33-57) at 25 years post Fontan. However, 95% (95 %CI 91-99) of patients without any of the identified risk factors were free from death or heart transplantation rate at 25 years post Fontan.

Conclusion: In conclusion, the occurrence of post-operative complications such as PLE, arrhythmia and ventricular dysfunction will likely precede the late demise of these patients.

Keywords: Congenital Heart Disease; Functional Single Ventricle; Heart Failure; Late death; Transplantation.