Chin Leng Poh1, Andrew Cochrane2, John C Galati3, Andrew Bullock4, David S Celermajer5, Tom Gentles6, Karin du Plessis7, David S Winlaw8, Tim Hornung6, Kirsten Finucane6, Yves d'Udekem9. 1. Murdoch Children's Research Institute, Melbourne, VIC, Australia Department of Paediatrics, Faculty of Medicine, University of Melbourne, Melbourne, VIC, Australia. 2. Department of Cardiothoracic Surgery, Monash Medical Centre, Monash University Faculty of Medicine, Melbourne, VIC, Australia. 3. Murdoch Children's Research Institute, Melbourne, VIC, Australia Department of Mathematics and Statistics, La Trobe University, Melbourne, VIC, Australia. 4. Children's Cardiac Centre, Princess Margaret Hospital for Children, Perth, WA, Australia. 5. Department of Medicine, The University of Sydney, Sydney, NSW, Australia Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia. 6. Green Lane Paediatric and Congenital Cardiac Service, Starship Children's Hospital Auckland, Auckland, New Zealand. 7. Murdoch Children's Research Institute, Melbourne, VIC, Australia. 8. Heart Centre for Children, The Children's Hospital at Westmead, Sydney, NSW, Australia. 9. Murdoch Children's Research Institute, Melbourne, VIC, Australia Department of Paediatrics, Faculty of Medicine, University of Melbourne, Melbourne, VIC, Australia The Royal Children's Hospital, Melbourne, VIC, Australia yves.dudekem@rch.org.au.
Abstract
OBJECTIVE: To investigate the benefits of a strategy of early Fontan conversion. METHODS: Using the Australia and New Zealand Fontan Registry, retrospective analysis of their long-term follow-up data was performed. RESULTS: Between 1990 and 2014, a total of 39 patients underwent surgical conversion in 6 centres at a median age of 23.8 years (IQR: 19.3-28.2), 18.7 ± 5.0 years post-Fontan. One centre tended to perform conversion earlier: interval since first documented arrhythmia 2.9 ± 4.0 vs 4.0 ± 4.2 years, average NYHA Class 2 ± 0.4 vs 3 ± 0.9 (P = 0.008), mean number of preop anti-arrhythmics 1 ± 0.4 vs 2 ± 0.6 (P = 0.05). Two patients underwent conversion to an extracardiac conduit only, while 36 had concomitant right atrial cryoablation, of which 16 also had pacemaker implantation. Nine patients suffered major cardiac-related complications (7 low output syndrome, 3 ECMO, 3 acute renal failure, one stroke) (2/17 from the early conversion centre and 7/22 of the others; P = 0.14). Four patients died in hospital (10.3%) and 4 late after a median of 0.9 years [95% confidence interval (CI): 0.5-1] after conversion. An additional 2 patients needed transplantation at 1 and 8.8 years after conversion, respectively. The 10-year freedom from heart transplantation was 86% (95% CI: 51-97%). Outcomes from the centre with an early conversion strategy were significantly better: 8-year freedom from death or heart transplantation was 86% (95% CI: 53-96) vs 51% (95% CI: 22-74; log-rank P = 0.007). Eight additional patients required pacemaker implantation and 5 had arrhythmia recurrence. CONCLUSIONS: Fontan conversion is associated with lasting survival outcomes up to 10 years after conversion. A strategy of surgical conversion at earlier stage of failure may be associated with better survival free from transplantation.
OBJECTIVE: To investigate the benefits of a strategy of early Fontan conversion. METHODS: Using the Australia and New Zealand Fontan Registry, retrospective analysis of their long-term follow-up data was performed. RESULTS: Between 1990 and 2014, a total of 39 patients underwent surgical conversion in 6 centres at a median age of 23.8 years (IQR: 19.3-28.2), 18.7 ± 5.0 years post-Fontan. One centre tended to perform conversion earlier: interval since first documented arrhythmia 2.9 ± 4.0 vs 4.0 ± 4.2 years, average NYHA Class 2 ± 0.4 vs 3 ± 0.9 (P = 0.008), mean number of preop anti-arrhythmics 1 ± 0.4 vs 2 ± 0.6 (P = 0.05). Two patients underwent conversion to an extracardiac conduit only, while 36 had concomitant right atrial cryoablation, of which 16 also had pacemaker implantation. Nine patients suffered major cardiac-related complications (7 low output syndrome, 3 ECMO, 3 acute renal failure, one stroke) (2/17 from the early conversion centre and 7/22 of the others; P = 0.14). Four patients died in hospital (10.3%) and 4 late after a median of 0.9 years [95% confidence interval (CI): 0.5-1] after conversion. An additional 2 patients needed transplantation at 1 and 8.8 years after conversion, respectively. The 10-year freedom from heart transplantation was 86% (95% CI: 51-97%). Outcomes from the centre with an early conversion strategy were significantly better: 8-year freedom from death or heart transplantation was 86% (95% CI: 53-96) vs 51% (95% CI: 22-74; log-rank P = 0.007). Eight additional patients required pacemaker implantation and 5 had arrhythmia recurrence. CONCLUSIONS: Fontan conversion is associated with lasting survival outcomes up to 10 years after conversion. A strategy of surgical conversion at earlier stage of failure may be associated with better survival free from transplantation.
Authors: Ganesh K Gnanappa; David S Celermajer; Gary F Sholler; Tom Gentles; David Winlaw; Yves d'Udekem; Julian Ayer Journal: Pediatr Cardiol Date: 2016-10-27 Impact factor: 1.655
Authors: John L Fitzgerald; Nicholas J Collins; James Leitch; Eugene Downar; Krishnakumar Nair; Nicholas Jackson Journal: HeartRhythm Case Rep Date: 2019-07-19