Impact of Fenestration Patency on Long-Term Fontan Outcomes

OBJECTIVES: Routine creation of a fenestration during Fontan completion is our policy to stabilize early circulation. This study evaluated its validity by examining the impact of fenestration patency at 1 year on long-term outcomes. METHODS: Among 112 patients who underwent fenestrated total cavopulmonary connection at 4 institutions, 105 with 1-year catheterization and ≥3 years of follow-up were analysed. Patients were classified by fenestration status at 1 year: patent (Group F, n = 43) and closed (Group C, n = 62). Preoperative and 1-year catheter data and long-term complications-Fontan-associated liver disease (FALD), protein-losing enteropathy (PLE), and catheter interventions excluding veno-venous collaterals-were compared. RESULTS: The median age at Fontan was 23 months (interquartile range [IQR]: 15-33). Preoperative haemodynamics showed no differences between the groups. At postoperative 1 year, systemic ventricular end-diastolic pressure (SVEDP) was higher in Group F (F: 8 [5-9] vs C: 6 [5-8] mmHg, P = .007). Protein-losing enteropathy was significantly more common in the Group C (F: 5.0% vs C: 27.0%, P = .038). In contrast, there was no significant difference in the cumulative incidence of FALD between the 2 groups (F: 12.5% vs C: 9.7%, P = .69). CONCLUSIONS: Although patent fenestration was associated with an increased SVEDP at 1 year postoperatively, it may improve long-term outcomes including PLE. As predicting eventual fenestration patency from preoperative data is difficult, our policy of routine fenestration creation appears to be a valid strategy. CLINICAL REGISTRATION NUMBER: Kitasato University, No. B23-130; February 7, 2024.