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. 2025 Nov 10:S1071-9164(25)00989-3.
doi: 10.1016/j.cardfail.2025.08.028. Online ahead of print.

Contemporary Waitlist and Post-Heart Transplant Outcomes for Fontan patients: An International Multi-Institutional Analysis

Shahnawaz Amdani  1 , Edo Bedzra  2 , Devin Koehl  3 , Ryan Cantor  3 , James K Kirklin  3 , Ryan L Kobayashi  4 , Natalie Shwaish  5 , Adam Putschoegl  6 , Steven D Zangwill  7 , Neha Bansal  8 , Nancy J Halnon  9
Affiliations

Contemporary Waitlist and Post-Heart Transplant Outcomes for Fontan patients: An International Multi-Institutional Analysis

Shahnawaz Amdani et al. J Card Fail. .

Abstract

Background: Pediatric patients with Fontan circulation represent a growing, high-risk cohort of heart transplant (HT) candidates. However, contemporary multi-institutional data on their waitlist and post-transplant outcomes are limited.

Methods: We conducted a retrospective analysis of children aged 2 to <18 years listed for HT in the Pediatric Heart Transplant Society database from 1993-2023. Patients were categorized as having Fontan circulation, non-Fontan congenital heart disease (CHD), or cardiomyopathy (CMP). Outcomes were analyzed across early (1993-2014) and current (2015-2023) eras. Cox proportional hazards models identified risk factors for waitlist mortality and post-transplant graft loss.

Results: Among 6,061 listed patients, 22.3% had Fontan circulation, 24.1% non-Fontan CHD, and 53.6% CMP. In the current era, 54.6% of CHD listings were Fontan patients. Fontan patients were significantly less likely to receive ventricular assist device (VAD) support at listing (2.6%) or transplant (4.7%) compared to CMP (16.4%, 29.5%; p<0.001). Waitlist survival has improved significantly, with no differences among diagnostic groups in the current era (p=0.109). However, Fontan patients had a twofold higher risk of post-transplant graft loss compared to CMP (HR 1.99, p<0.0001), and experienced higher rates of post-transplant infection and malignancy (p<0.05). Among Fontan patients, VAD-supported individuals had similar post-transplant survival to those without mechanical support and significantly better survival than those supported with ECMO (p<0.0001).

Conclusions: Fontan patients now represent the largest CHD subgroup listed for HT. While waitlist outcomes have improved, post-transplant outcomes remain inferior. Selective and timely use of VAD support may improve survival in this high-risk population.

Keywords: Fontan; cardiomyopathy; congenital heart disease; graft loss; heart transplant; pediatric; transplant outcomes; waitlist outcomes.

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Conflict of interest statement

Declaration of competing interest Dr Amdani is the site principal investigator for a multicenter study led by the University of Michigan and site principal investigator for FUEL-2 (Fontan Udenafil Exercise Longitudinal Assessment Trial, Mezzion) and has received no salary support. J.K.K receives royalties related to IT software development in registry database design developed at and licensed from the University of Alabama at Birmingham, payments from Kirklin Solutions Co. database development and analytics and partial salary support in his role as Director of the data center for STS Intemacs/Pedimacs Registries; serves as Chair of DSMB for Xeltis cardiac conduit clinical trial and Chair of DSMB for Carmat TAH clinical trial. No other author(s) have any disclosures to report.

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