“Fontan Conduit Stent-Angioplasty and Progression of Fontan-Associated Liver Disease”

Abstract

Abstract Background: Patients with Fontan circulation insidiously develop congestive hepatopathy related to chronically reduced cardiac output and central venous hypertension, also known as Fontan-associated liver disease (FALD). Fontan pathway obstruction is increasingly detected and may accelerate FALD. The impact of conduit stent-angioplasty on FALD is unknown. Methods: Retrospective, single-center review of patients with Fontan circulation who underwent conduit stent-angioplasty at cardiac catheterization over 5-year period. Demographics and cardiac histories were reviewed. Labs, liver ultrasound elastography, echocardiogram, hemodynamic and angiographic data at catheterization were recorded pre- and post-stent-angioplasty. Primary outcome was change in hepatic function via MELD-XI scores and liver stiffness (kPa), with secondary outcomes of ventricular function, BNP, and repeat catheterization hemodynamics. Results: 33 patients underwent Fontan conduit stent angioplasty, 19.3 ±7.0 years from Fontan operation. Original conduit diameter was 19.1 ±1.9 mm. Prior to angioplasty, conduit size was reduced to a cross-sectional area 132 (91, 173) mm2 and increased to 314 (255, 363) mm2 post-stent. Subjects' baseline median MELD-XI of 11 (9, 12) increased to 12 (9, 13) at 19 ± 15.5 months post-angioplasty (n=22, p= 0.053). There was no significant change in liver stiffness at 12.1 ±8.9 months post-angioplasty (n=15, p= 0.13). Median total bilirubin significantly increased (1.4 [0.9, 1.8]), from baseline 1.1 [0.7, 1.5], p= 0.04), as did median BNP (41 [0, 148] from baseline 34 [15, 79]; p= 0.02). There were no significant changes in ventricular function or repeat invasive hemodynamics (n=8 subjects). Conclusions: Mid-term follow-up of Fontan subjects post-conduit stent angioplasty did not show improvements in non-invasive markers of FALD.