title: Unsatisfactory Early and Late Outcomes After Fontan Surgery Delayed to Adolescence and Adulthood.
abstract: The ideal age to perform the Fontan procedure is still unknown. The aim of this study is to determine outcomes after Fontan surgery delayed to adolescence and adulthood in Australia and New Zealand. Patients who had undergone a Fontan procedure at 15 years of age or older were identified in the 1133 patients registered in the Australia and New Zealand Fontan Registry until December 2012. A total of 45 patients underwent the following Fontan procedure at a median age of 18.3 years (16-21 years): 24 atriopulmonary connections, 10 lateral tunnel, and 11 extracardiac conduits. Hospital mortality was 13% (6 of 45). After a mean follow-up of 15.5 ± 9 years, there were 8 late deaths. Survival rates after 10, 20, and 25 years were 79% (95% CI: 64-89), 70% (95% CI: 51-83), and 70% (95% CI: 51-83), respectively. Freedom from Fontan failure (death, heart transplantation, Fontan takedown, protein-losing enteropathy, and poor functional status) after 10 and 20 years was 63% (95% CI: 47-76) and 35% (95% CI: 19-52), respectively. Patients with a single left ventricle had a lower risk of failure (hazard ratio = 0.25, 95% CI: 0.10-0.59; P = 0.002). Arrhythmias developed in 29 patients after a median of 0.1 years (0-9.3 years) and 10 required a permanent pacemaker. Freedom from all adverse events at 10 years was 30% (95% CI: 16-45). Outcomes of the Fontan procedure in adolescents and adults are poor, with disproportionately high hospital mortality and late adverse events. The Fontan procedure should not be delayed to adolescence and adulthood and should be performed electively in childhood.
authors.affiliation: Department of Medicine, The University of Notre Dame, Sydney, New South Wales, Australia.
authors.forename: Victoria
authors.initials: V
authors.lastname: Forsdick
authors.affiliation: Department of Cardiac Surgery, Royal Children׳s Hospital, Melbourne, Australia; Heart Research, Murdoch Childrens Research Institute, Melbourne, Australia; Department of Pediatrics of the University of Melbourne, Melbourne, Australia.
authors.forename: Ajay J
authors.initials: AJ
authors.lastname: Iyengar
authors.affiliation: Department of Pediatrics of the University of Melbourne, Melbourne, Australia.
authors.forename: Thomas
authors.initials: T
authors.lastname: Carins
authors.affiliation: Green Lane Paediatric and Congenital Cardiac Service, Starship Children׳s Hospital, Auckland, New Zealand.
authors.forename: Thomas L
authors.initials: TL
authors.lastname: Gentles
authors.affiliation: Department of Cardiology, Royal Children׳s Hospital, Melbourne, Australia.
authors.forename: Robert G
authors.initials: RG
authors.lastname: Weintraub
authors.affiliation: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia; Department of Medicine, The University of Sydney, Sydney, New South Wales, Australia.
authors.forename: David S
authors.initials: DS
authors.lastname: Celermajer
authors.affiliation: Department of Cardiac Surgery, Royal Children׳s Hospital, Melbourne, Australia; Heart Research, Murdoch Childrens Research Institute, Melbourne, Australia; Department of Pediatrics of the University of Melbourne, Melbourne, Australia. Electronic address: yves.dudekem@rch.org.au.
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
mesh_terms: D000293:Adolescent; D000367:Age Factors; D001315:Australia; D005260:Female; D018729:Fontan Procedure / Q000009:adverse effects* / Q000401:mortality; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D017052:Hospital Mortality; D006801:Humans; D008297:Male; D009520:New Zealand; D018579:Patient Selection; D011183:Postoperative Complications / Q000401:mortality / Q000628:therapy; D012042:Registries; D018570:Risk Assessment; D012307:Risk Factors; D013997:Time Factors; D061665:Time-to-Treatment; D017211:Treatment Failure; D055815:Young Adult
keywords: congenital heart disease; follow-up study; fontan; single ventricle