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

• Published in: International journal of cardiology. Heart & vasculature 2021-08, Vol.35, p.100825-100825, Article 100825
• Main Authors: Poh, Chin L., Cordina, Rachael L., Iyengar, Ajay J., Zannino, Diana, Grigg, Leeanne E., Wheaton, Gavin R., Bullock, Andrew, Ayer, Julian, Alphonso, Nelson, Gentles, Thomas L., Celermajer, David S., d'Udekem, Yves
• Format: Article
• Language: English
• Subjects: Congenital Heart Disease; Functional Single Ventricle; Heart Failure; Late death; Original Paper; Transplantation
• ISSN: 2352-9067; 2352-9067
• DOI: 10.1016/j.ijcha.2021.100825

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