Chin Leng Poh1, Diana Zannino2, Robert G Weintraub3, David S Winlaw4, Leeanne E Grigg5, Rachael Cordina6, Tim Hornung7, Andrew Bullock8, Robert N Justo9, Thomas L Gentles7, Charlotte Verrall4, Karin du Plessis10, David S Celermajer6, Yves d'Udekem11. 1. Department of Paediatrics, University of Melbourne, Melbourne, VIC, Australia; Murdoch Childrens Research Institute, Heart Research Group, Melbourne, VIC, Australia. 2. Murdoch Childrens Research Institute, Clinical Epidemiology & Biostatistics, Melbourne, VIC, Australia. 3. Department of Cardiology, Royal Children's Hospital, Melbourne, VIC, Australia. 4. Department of Paediatric Cardiac Surgery, The Children's Hospital at Westmead, Sydney, NSW, Australia. 5. Department of Cardiology, Royal Melbourne Hospital, Melbourne, VIC, Australia. 6. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia. 7. Department of Paediatric Cardiology, Starship Children's Hospital, Auckland, New Zealand. 8. Princess Margaret Hospital for Children and Royal Perth Hospital, Perth, WA, Australia. 9. Queensland Paediatric and Congenital Cardiac Service, Brisbane, Lady Cilento Children's Hospital, Brisbane, Qld, Australia. 10. Murdoch Childrens Research Institute, Heart Research Group, Melbourne, VIC, Australia. 11. Department of Paediatrics, University of Melbourne, Melbourne, VIC, Australia; Murdoch Childrens Research Institute, Heart Research Group, Melbourne, VIC, Australia; Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, VIC, Australia. Electronic address: yves.dudekem@rch.org.au.
Abstract
OBJECTIVE: To review our experience of patients with an atrio-pulmonary Fontan circulation to determine their long-term outcomes. METHODS AND RESULTS: A retrospective analysis of long-term follow-up data using the Australia and New Zealand Fontan Registry was performed. There were 215 patients surviving hospital discharge after an atrio-pulmonary Fontan completion. A total of 163 patients were alive at latest follow-up, with 52 deaths. Twelve patients had required heart transplantation and 95 had Fontan failure (death, transplantation, Fontan takedown, Fontan conversion, severe systemic ventricular dysfunction or NYHA≥3). Twenty-eight year freedom from death, death and transplantation and Fontan failure were 69% (95% CI 61-78%), 64% (95% CI 56-74%) and 45% (95% CI 36-55%) respectively. One hundred and thirty patients developed atrial arrhythmias. Freedom from arrhythmia at 28years post Fontan was 22.9% (95% CI: 15.1-30.8). Development of arrhythmia increased the likelihood of death (HR:2.97, 95%CI 1.50-5.81; p=0.002), death and heart transplantation (HR:3.11, 95%CI 1.64-5.87; p<0.001) and Fontan failure (HR:4.78 95%CI 2.95-7.74; p<0.001). There were 42 patients who had thromboembolic events, of which only 12 were therapeutically anti-coagulated. Two-thirds of the surviving patients (86/126) with an intact atrio-pulmonary Fontan were regularly reviewed. Patients receiving follow-up care with general cardiologists without specialised training were more likely to face Fontan failure than those managed by cardiologists with specialist training in congenital heart disease (HR: 1.94, 95% CI 1.16-3.24; p=0.02). The majority of the surviving patients (81/86) remained physically active and almost two-thirds (54/86) were currently employed. CONCLUSION: Two-thirds of the patients who underwent a classical atrio-pulmonary Fontan are still alive 3 decades later. The majority are affected by the burden of arrhythmias but remain functionally active today. These challenging patients should only receive follow-up care from cardiologists with specialised training.
OBJECTIVE: To review our experience of patients with an atrio-pulmonary Fontan circulation to determine their long-term outcomes. METHODS AND RESULTS: A retrospective analysis of long-term follow-up data using the Australia and New Zealand Fontan Registry was performed. There were 215 patients surviving hospital discharge after an atrio-pulmonary Fontan completion. A total of 163 patients were alive at latest follow-up, with 52 deaths. Twelve patients had required heart transplantation and 95 had Fontan failure (death, transplantation, Fontan takedown, Fontan conversion, severe systemic ventricular dysfunction or NYHA≥3). Twenty-eight year freedom from death, death and transplantation and Fontan failure were 69% (95% CI 61-78%), 64% (95% CI 56-74%) and 45% (95% CI 36-55%) respectively. One hundred and thirty patients developed atrial arrhythmias. Freedom from arrhythmia at 28years post Fontan was 22.9% (95% CI: 15.1-30.8). Development of arrhythmia increased the likelihood of death (HR:2.97, 95%CI 1.50-5.81; p=0.002), death and heart transplantation (HR:3.11, 95%CI 1.64-5.87; p<0.001) and Fontan failure (HR:4.78 95%CI 2.95-7.74; p<0.001). There were 42 patients who had thromboembolic events, of which only 12 were therapeutically anti-coagulated. Two-thirds of the surviving patients (86/126) with an intact atrio-pulmonary Fontan were regularly reviewed. Patients receiving follow-up care with general cardiologists without specialised training were more likely to face Fontan failure than those managed by cardiologists with specialist training in congenital heart disease (HR: 1.94, 95% CI 1.16-3.24; p=0.02). The majority of the surviving patients (81/86) remained physically active and almost two-thirds (54/86) were currently employed. CONCLUSION: Two-thirds of the patients who underwent a classical atrio-pulmonary Fontan are still alive 3 decades later. The majority are affected by the burden of arrhythmias but remain functionally active today. These challenging patients should only receive follow-up care from cardiologists with specialised training.
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