Abstract / Summary
Expanded polytetrafluoroethylene (e-PTFE) conduits have been widely used in Fontan procedures; however, the progression of conduit stenosis due to long-term calcification, indications for reintervention, and postoperative clinical outcomes after surgical revision remain incompletely characterized. This study aimed to characterize the clinical and morphological features, temporal progression, treatment course, and postoperative outcomes of patients who required surgical revision for severely calcified Fontan conduit stenosis. We retrospectively reviewed 415 patients who underwent primary Fontan procedures using e-PTFE conduits at our institution between January 1990 and May 2025. Among 180 patients followed for more than 20 years, seven patients who required surgical revision for severe calcification-related conduit stenosis were included. Clinical characteristics, serial imaging findings, cardiac catheterization data, treatment course, operative findings, pathological findings, and pre- and postoperative MELD-XI and APRI scores were analyzed. Among the 180 patients followed for more than 20 years, seven required surgical revision for severe calcification-related conduit stenosis. The median interval from initial Fontan procedure to first recognition of conduit stenosis was 24 years [interquartile range (IQR): 23–27], and to surgical revision was 28 [26–31] years. The median duration from stenosis recognition to surgery was 3.3 [1.6–4.5] years. During this period, patients were managed with antithrombotic therapy, or balloon angioplasty; however, stenosis progressed in all cases. All patients demonstrated > 50% morphological stenosis, while resting pressure gradients across the conduit remained low. All patients underwent conduit replacement via redo sternotomy, with no in-hospital mortality. MELD-XI and APRI scores were stable or improved in most patients, although improvement was limited in those with advanced end-organ dysfunction. In this single-center long-term cohort, severe calcification-related stenosis of e-PTFE Fontan conduits requiring surgical revision was observed in a small subset of patients more than two decades after the primary Fontan procedure. Substantial anatomical stenosis may be present despite low resting pressure gradients, supporting lifelong multimodality imaging surveillance. Surgical conduit replacement was feasible with no in-hospital mortality in this series, although patients with established Fontan-related organ dysfunction may have limited postoperative recovery.