The Fontan Pathway: Change in Dimension and Catheter-Based Intervention over Time

An unobstructed Fontan pathway is essential for optimal hemodynamics. We hypothesize that more extracardiac conduit (ECC) Fontan pathways develop obstruction compared to lateral tunnel (LT) Fontans and that the dilation typically observed in LTs results in similar mid-term clinical outcomes. A single-center, retrospective study was done including all Fontan cardiac catheterizations from 2006 to 2019. Angiography and medical records were reviewed to define Fontan pathway dimensions, interventions, and clinical outcomes. 232 patients underwent cardiac catheterization, where 60% were ECCs and 30% LTs. The minimum cross-sectional area (CSA) of ECCs was significantly smaller than LTs and LTs dilated over time. 13% of patients had Fontan pathway stenting at a median age of 16.2 years. The minimum CSA for patients who underwent intervention was significantly smaller than patients who did not. Lower weight at Fontan surgery was associated with intervention on the Fontan pathway, with a threshold weight of 15 kg for patients with an ECC. The median follow-up was 3.3 years. Patients who had Fontan pathway intervention were not more likely to experience the composite adverse clinical outcome. LTs were more likely than ECCs to have worse clinical outcome, when liver fibrosis was included. This is the first study to describe angiographic dimensions of the Fontan pathway in a large number of patients over time. ECCs tend to become stenotic. Lower weight at Fontan surgery is a potential risk for Fontan pathway intervention. LTs may experience worse clinical outcomes in follow-up. This information can help inform the optimal timing and method of post-Fontan surveillance.