Mark Dennis1, Diana Zannino2, Karin du Plessis2, Andrew Bullock3, Patrick J S Disney4, Dorothy J Radford5, Tim Hornung6, Leeanne Grigg7, Rachael Cordina1, Yves d'Udekem8, David S Celermajer9. 1. Sydney Medical School, University of Sydney, Camperdown, Sydney, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Missenden Road, Sydney, Australia. 2. Murdoch Children's Research Institute, Melbourne, Australia. 3. Children's Cardiac Centre, Princess Margaret Hospital for Children, Perth, Australia. 4. Department of Cardiovascular Services, Royal Adelaide Hospital, Adelaide, Australia. 5. Adult Congenital Heart Unit, The Prince Charles Hospital, Brisbane, Australia. 6. Green Lane Paediatric and Congenital Cardiac Service, Starship Children's Hospital Auckland, Auckland, New Zealand. 7. Department of Cardiology, The Royal Melbourne Hospital, Melbourne, Australia. 8. Murdoch Children's Research Institute, Melbourne, Australia; Departments of Cardiac Surgery and Cardiology, Royal Children's Hospital, Melbourne, Australia. 9. Sydney Medical School, University of Sydney, Camperdown, Sydney, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Missenden Road, Sydney, Australia. Electronic address: david.celermajer@sydney.edu.au.
Abstract
BACKGROUND: Long-term outcomes of Fontan patients who survive to age ≥16 years have not been well characterized. The Australian and New Zealand Fontan Registry (ANZFR) provides a unique opportunity to understand survival and complication rates in Fontan patients who transition to adult congenital heart disease centers. OBJECTIVES: This study sought to describe the survival and complications of adult patients who have had a Fontan procedure. METHODS: The study analyzed outcomes in patients ≥16 years of age who were prospectively enrolled in the ANZFR. RESULTS: Data from all 683 adult survivors from the ANZFR were analyzed. Mortality status was confirmed from the National Death Index. There were 201 atriopulmonary (AP) connections and 482 total cavopulmonary connections (249 lateral tunnels and 233 extracardiac conduits). For these subjects, the survival rate at age 30 years was 90% (95% CI: 87% to 93%), and it was 80% (95% CI: 75% to 87%) at 40 years of age. Survival at age 30 years was significantly worse for the patients with AP connections (p = 0.03). At latest follow-up, only 53% of patients were in New York Heart Association functional class I. After the age of 16 years, 136 (20%) had experienced at least 1 new arrhythmia, 42 (6%) required a permanent pacemaker, 45 (7%) had a thromboembolic event, and 135 (21%) required a surgical reintervention. Only 41% (95% CI: 33% to 51%) of Fontan patients were free of serious adverse events at 40 years of age. CONCLUSIONS: This comprehensively followed cohort showed that a variety of morbid complications is common in Fontan adults, and that there is a substantial incidence of premature death, particularly in patients with AP connections.
BACKGROUND: Long-term outcomes of Fontan patients who survive to age ≥16 years have not been well characterized. The Australian and New Zealand Fontan Registry (ANZFR) provides a unique opportunity to understand survival and complication rates in Fontan patients who transition to adult congenital heart disease centers. OBJECTIVES: This study sought to describe the survival and complications of adult patients who have had a Fontan procedure. METHODS: The study analyzed outcomes in patients ≥16 years of age who were prospectively enrolled in the ANZFR. RESULTS: Data from all 683 adult survivors from the ANZFR were analyzed. Mortality status was confirmed from the National Death Index. There were 201 atriopulmonary (AP) connections and 482 total cavopulmonary connections (249 lateral tunnels and 233 extracardiac conduits). For these subjects, the survival rate at age 30 years was 90% (95% CI: 87% to 93%), and it was 80% (95% CI: 75% to 87%) at 40 years of age. Survival at age 30 years was significantly worse for the patients with AP connections (p = 0.03). At latest follow-up, only 53% of patients were in New York Heart Association functional class I. After the age of 16 years, 136 (20%) had experienced at least 1 new arrhythmia, 42 (6%) required a permanent pacemaker, 45 (7%) had a thromboembolic event, and 135 (21%) required a surgical reintervention. Only 41% (95% CI: 33% to 51%) of Fontan patients were free of serious adverse events at 40 years of age. CONCLUSIONS: This comprehensively followed cohort showed that a variety of morbid complications is common in Fontan adults, and that there is a substantial incidence of premature death, particularly in patients with AP connections.
Authors: David J Goldberg; Victor Zak; Bryan H Goldstein; Kurt R Schumacher; Jonathan Rhodes; Daniel J Penny; Christopher J Petit; Salil Ginde; Shaji C Menon; Seong-Ho Kim; Gi Beom Kim; Todd T Nowlen; Michael V DiMaria; Benjamin P Frischhertz; Jonathan B Wagner; Kimberly E McHugh; Brian W McCrindle; Amanda J Shillingford; Arash A Sabati; Anji T Yetman; Anitha S John; Marc E Richmond; Matthew D Files; R Mark Payne; Andrew S Mackie; Christopher K Davis; Shabana Shahanavaz; Kevin D Hill; Ruchira Garg; Jeffrey P Jacobs; Michelle S Hamstra; Stacy Woyciechowski; Kathleen A Rathge; Michael G McBride; Peter C Frommelt; Mark W Russell; Elaine M Urbina; James L Yeager; Victoria L Pemberton; Mario P Stylianou; Gail D Pearson; Stephen M Paridon Journal: Circulation Date: 2019-11-17 Impact factor: 29.690
Authors: Anita Saraf; Christine De Staercke; Ian Everitt; Alice Haouzi; Yi-An Ko; Staci Jennings; Jonathan H Kim; Fred H Rodriguez; Andreas P Kalogeropoulos; Arshed Quyyumi; Wendy Book Journal: Int J Cardiol Date: 2020-01-09 Impact factor: 4.164
Authors: Sheyanth Mohanakumar; Benjamin Kelly; Aida Luiza Ribeiro Turquetto; Mathias Alstrup; Luciana Patrick Amato; Milena Schiezari Ru Barnabe; João Bruno Dias Silveira; Fernando Amaral; Paulo Henrique Manso; Marcelo Biscegli Jatene; Vibeke Elisabeth Hjortdal Journal: Physiol Rep Date: 2021-06
Authors: Jack Rubinstein; Jessica G Woo; Anastacia M Garcia; Tarek Alsaied; Jia Li; Per Kristian Lunde; Ryan A Moore; Martin Laasmaa; Amanda Sammons; Wayne A Mays; Shelley D Miyamoto; William E Louch; Gruschen R Veldtman Journal: Pediatr Cardiol Date: 2020-08-07 Impact factor: 1.655