Contemporary outcomes of the Fontan operation: An Australian and New Zealand Fontan Registry study.

Authors

Michael Daley, Igor E Konstantinov, Supreet Marathe, Tim Hornung, Patrick Disney, Nelson Alphonso, Jelena Saundankar, Gavin Wheaton, Leeanne Grigg, Vishva Wijesekera, Ajay Iyengar, Julian Ayer, Rachael Cordina, Yves d'Udekem

Published in

JTCVS open. Volume 32. Pages 101833. Epub May 04, 2026.

Abstract

We sought to review the impact of extracardiac conduit size and risk factors on the long-term outcomes of the contemporary Fontan procedure: lateral tunnel and extracardiac conduits.
Data were collected from a binational registry. Composite outcome of late adverse events included death, transplant, Fontan failure, supraventricular tachycardia (SVT), thromboembolism, or pacemaker implantation.
Transplant-free survival and freedom from Fontan failure was 85% (95% CI, 80%-89%) and 75% (95% CI, 70%-80%) at 30 years, respectively. Extracardiac conduit sizes were available in 1075 patients, with 18 mm (n = 596) and 20 mm (n = 355) being the most common. There was no difference in transplant-free survival, Fontan failure, or late adverse events between patients who received extracardiac conduits with smaller or larger conduits (≤18 mm vs ≥20 mm, P = .41, P = .50, and P = .27, respectively). Only 8 patients (<1%) underwent conduit upsizing during follow-up, all concurrently with another intervention. Lateral tunnel, isomerism, and fenestration were associated with sustained SVT on multivariable analysis (hazard ratio [HR], 2.0, P = .002; HR, 2.4, P < .001; HR, 1.7, P = .013, respectively). Only Fontan fenestration and isomerism were associated with the incidence of thromboembolic events (n = 161) on multivariable analysis (HR, 1.5, P = .021 and HR, 1.4, P = .025, respectively). Landmark analysis was performed from 16 years of age in patients without Fontan failure (n = 813). Transplant-free survival and freedom from Fontan failure were 87% (95% CI, 81%-91%) and 80% (95% CI, 72%-85%) at 20 years from this time point, respectively.
Despite recent suggestions, extracardiac conduit sizes do not appear to affect long-term clinical outcomes. Patients with lateral tunnel have a greater incidence of sustained SVT, and Fontan fenestration may increase incidence of late adverse events.

PMID:
42604323
Bibliographic data and abstract were imported from PubMed on 16 Aug 2026.

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