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Impact Of Type Of Fontan Operation (Extracardiac Versus Lateral Tunnel) On The Occurrence Of Fontan Associated Liver Disease
Aanya Chopra, BS¹, Ryan Biskowitz¹, Samuel Zhang¹, Micheal McCormick¹, John L. Myers, MD², Vincent Aluquin, MD², Bryan Stefek, MD², Joseph Brian Clark, MD³, Allen Kunselman¹, Karthik Ramakrishnan, MD².
¹The Penn State College of Medicine, Hershey, PA, USA, ²Penn State Health Golisano Children's Hospital, Hershey, PA, USA, ³Duke Children’s Pediatric and Congenital Heart Center, Durham, NC, USA.

Objective: Fontan-associated liver disease (FALD) is a recognized long-term complication after the Fontan operation. We evaluated risk factors for FALD, focusing on the impact of Fontan type (extracardiac vs. lateral tunnel). Methods: We retrospectively studied patients who underwent Fontan completion from 2008–2019. The primary outcome was the development of FALD. Secondary outcomes included mortality and transplantation. Group comparisons used chi-square/Fisher’s exact tests. Kaplan-Meier analysis and Cox proportional hazards modeling assessed time to FALD and associated risk factors. Results: Eighty-nine patients were included (25 extracardiac, 64 lateral tunnel). The extracardiac group was older at Fontan (4.36 vs. 3.26 years, p=0.03) and had higher rates of syndromic diagnoses (p=0.007) and heterotaxy (p=0.005). 64% (16/25) of the extracardiac conduits were 18mm and 32% (8/25) were 20mm. There was only one (4%) 16mm conduit used in this cohort. Median follow-up was 9 years (IQR 6–12). FALD was documented in 24 patients (27%) (9 extracardiac (37.5%), 15 lateral tunnel (62.5%). Median time to FALD was shorter in the extracardiac group (11.7 vs. 15.3 years p=0. 004. Figure 1). On multivariable analysis, extracardiac Fontan was associated with a significantly higher risk of FALD (HR 3.31, 95% CI 1.41–7.79, p=0.006). Conclusions: Survival and transplant rates were similar between groups. However, extracardiac Fontan was associated with significantly increased risk of FALD. FALD also occurred earlier in the extracardiac Fontan cohort. These findings suggest the lateral tunnel approach may not only confer protection against FALD but may also delay development of FALD.
Figure 1. Freedom from FALD

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