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

• Published in: Pediatric cardiology Vol. 46; no. 2; pp. 372 - 378
• Main Authors: Kavin, Umakanthan, Shahrier, Aniqa, Bandisode, Varsha M., Chowdhury, Shahryar M., Rhodes, John F., Gaydos, Stephanie S.
• Format: Journal Article
• Language: English
• Published: New York Springer US 01.02.2025
• Subjects: Adolescent; Angioplasty - methods; Cardiac Catheterization; Cardiac Surgery; Cardiology; Child; Child, Preschool; Disease Progression; Elasticity Imaging Techniques; Female; Follow-Up Studies; Fontan Procedure - adverse effects; Heart Defects, Congenital - surgery; Humans; Liver Diseases - diagnosis; Liver Diseases - etiology; Liver Diseases - physiopathology; Male; Medicine; Medicine & Public Health; Postoperative Complications; Retrospective Studies; Stents; Treatment Outcome; Vascular Surgery; Young Adult; Stent angioplasty; Liver disease; Fontan
• ISSN: 0172-0643; 1432-1971; 1432-1971
• DOI: 10.1007/s00246-024-03426-8

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. 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. 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) mm 2 and increased to 314 (255, 363) mm 2 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). Mid-term follow-up of Fontan subjects post-conduit stent angioplasty did not show improvements in non-invasive markers of FALD.