Thomas A Carins1, William Y Shi2, Ajay J Iyengar2, Ashley Nisbet3, Victoria Forsdick4, Diana Zannino5, Thomas Gentles6, Dorothy J Radford3, Robert Justo7, David S Celermajer8, Andrew Bullock9, David Winlaw10, Gavin Wheaton11, Leeanne Grigg12, Yves d'Udekem13. 1. Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia. 2. Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; The Murdoch Childrens Research Institute, Melbourne, Australia. 3. Department of Cardiology, The Prince Charles Hospital, Brisbane, Australia. 4. Department of Medicine, The University of Notre Dame, Sydney, Australia. 5. The Murdoch Childrens Research Institute, Melbourne, Australia. 6. Green Lane Congenital Cardiac Service, Starship Children's Hospital, Auckland, New Zealand. 7. Queensland Paediatric Cardiac Service, Lady Cilento Hospital, Brisbane, Queensland, Australia. 8. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia. 9. Paediatric Cardiology, Princess Margaret Hospital for Children, Perth, Australia. 10. The Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia. 11. Department of Cardiology, Women's and Children's Hospital, Adelaide, Australia. 12. Department of Cardiology, Royal Melbourne Hospital, Melbourne, Australia. 13. Department of Cardiac Surgery, The Royal Children's Hospital, Melbourne, Australia; Department of Paediatrics, University of Melbourne, Melbourne, Australia; The Murdoch Childrens Research Institute, Melbourne, Australia. Electronic address: yves.dudekem@rch.org.au.
Abstract
OBJECTIVES: Patients living with a Fontan circulation are prone to develop arrhythmias. However, their prognostic impact has been seldom studied. As such, we aimed to determine the incidence and predictors of arrhythmias after the Fontan procedure and the long-term outcomes after the first onset of arrhythmias. METHODS: Of the 1034 patients who have undergone a Fontan procedure as recorded in the Australian and New Zealand Fontan Registry, we identified those in whom a tachyarrhythmia or bradyarrhythmia developed. We evaluated the incidence and predictors of developing arrhythmias and their prognostic impact on late outcomes. RESULTS: Arrhythmia developed in 195 patients. Tachyarrhythmia was present in 162 patients, bradyarrhythmia was present in 74 patients, and both forms were present in 41 patients. At 20 years, freedom from any arrhythmia, tachyarrhythmia, and bradyarrhythmia was 66% (95% confidence interval [CI], 59-72), 69% (95% CI, 62-75), and 85% (95% CI, 80-90), respectively. On multivariable analyses, patients with an extracardiac Fontan (hazard ratio [HR], 0.23; 95% CI, 0.10-0.51; P < .001) were less likely to develop an arrhythmia, whereas those with left atrial (HR, 3.18; 95% CI, 1.45-6.95; P = .004) and right atrial (HR, 4.00; 95% CI, 2.41-6.61; P < .001) isomerism were more likely to have an arrhythmia. After onset of any arrhythmia (tachyarrhythmia or bradyarrhythmia), 10- and 15-year survivals were 74% (65%-83%) and 70% (60%-80%), respectively, and freedom from Fontan failure was 55% (44%-64%) and 44% (32%-56%), respectively. The development of any arrhythmia (HR, 2.20; 95% CI, 1-44-3.34; P < .001), tachyarrhythmia (HR, 2.56; 95% CI, 1.60-4.11; P < .001), and bradyarrhythmia (HR, 1.85; 95% CI, 1.16-2.95; P = .01) were all independent predictors of late Fontan failure on multivariable analyses. CONCLUSIONS: The development of an arrhythmia is associated with a heightened risk of subsequent failure of the Fontan circulation.
OBJECTIVES:Patients living with a Fontan circulation are prone to develop arrhythmias. However, their prognostic impact has been seldom studied. As such, we aimed to determine the incidence and predictors of arrhythmias after the Fontan procedure and the long-term outcomes after the first onset of arrhythmias. METHODS: Of the 1034 patients who have undergone a Fontan procedure as recorded in the Australian and New Zealand Fontan Registry, we identified those in whom a tachyarrhythmia or bradyarrhythmia developed. We evaluated the incidence and predictors of developing arrhythmias and their prognostic impact on late outcomes. RESULTS:Arrhythmia developed in 195 patients. Tachyarrhythmia was present in 162 patients, bradyarrhythmia was present in 74 patients, and both forms were present in 41 patients. At 20 years, freedom from any arrhythmia, tachyarrhythmia, and bradyarrhythmia was 66% (95% confidence interval [CI], 59-72), 69% (95% CI, 62-75), and 85% (95% CI, 80-90), respectively. On multivariable analyses, patients with an extracardiac Fontan (hazard ratio [HR], 0.23; 95% CI, 0.10-0.51; P < .001) were less likely to develop an arrhythmia, whereas those with left atrial (HR, 3.18; 95% CI, 1.45-6.95; P = .004) and right atrial (HR, 4.00; 95% CI, 2.41-6.61; P < .001) isomerism were more likely to have an arrhythmia. After onset of any arrhythmia (tachyarrhythmia or bradyarrhythmia), 10- and 15-year survivals were 74% (65%-83%) and 70% (60%-80%), respectively, and freedom from Fontan failure was 55% (44%-64%) and 44% (32%-56%), respectively. The development of any arrhythmia (HR, 2.20; 95% CI, 1-44-3.34; P < .001), tachyarrhythmia (HR, 2.56; 95% CI, 1.60-4.11; P < .001), and bradyarrhythmia (HR, 1.85; 95% CI, 1.16-2.95; P = .01) were all independent predictors of late Fontan failure on multivariable analyses. CONCLUSIONS: The development of an arrhythmia is associated with a heightened risk of subsequent failure of the Fontan circulation.
Authors: Jane W Newburger; Lynn A Sleeper; J William Gaynor; Danielle Hollenbeck-Pringle; Peter C Frommelt; Jennifer S Li; William T Mahle; Ismee A Williams; Andrew M Atz; Kristin M Burns; Shan Chen; James Cnota; Carolyn Dunbar-Masterson; Nancy S Ghanayem; Caren S Goldberg; Jeffrey P Jacobs; Alan B Lewis; Seema Mital; Christian Pizarro; Aaron Eckhauser; Paul Stark; Richard G Ohye Journal: Circulation Date: 2018-02-01 Impact factor: 29.690
Authors: Saji Oommen; Susana Cantero Peral; Muhammad Y Qureshi; Kimberly A Holst; Harold M Burkhart; Matthew A Hathcock; Walter K Kremers; Emma B Brandt; Brandon T Larsen; Joseph A Dearani; Brooks S Edwards; Joseph J Maleszewski; Timothy J Nelson Journal: Cell Transplant Date: 2022 Jan-Dec Impact factor: 4.139